Management of retinoblastoma

Dr Roopsi Sharma

DNB JR

MAX Hospital Saket

STAGING and CLASSIFICATION

bull ReesendashEllsworth system of classifying retinoblastoma (1964)

bull Predicts the outcome after EBRT

Based on

noof lesion size of lesion

Location of lesion and viterous seeding

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group I Very favorableASolitary tumor less than 4 DD at or behind the

equator B Multiple tumors none larger than 4 DD all at or behind the

equatorGroup II Favorable A Solitary tumor 4ndash10 DD at or behind the equator B Multiple tumors 4ndash10 DD all at or behind the equator

Group III Doubtful A Any lesion anterior to the equator B Solitary tumors larger than 10 DD behind the equator

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group IV Unfavorable

A Multiple tumors some larger than 10 DD

BAny lesion extending anterior to the oraserrata

Group V Very unfavorable

A Massive tumors involving more than half of the retina

BVitreous seeding

RE system DEMERITS

bull Doesnrsquot take into account retinal detachment or subretinal

tumor seeding prognostic factors for vision preservation

bull Small anterior tumors were placed in group 3 or 4 because

they were not usually controlled by ExRT technique in use

at that time but they are quite responsive to modern RT

techniques cryotherapy or RT plaque therapy

bull Vitreous seeding classified as group 5b although local

vitreous seeding can often be treated with RT plaque

International classification of retinoblastoma- 2006

Group A small intraretinal tumors away from the disc and foveola

bull No tumor greater than 3 mm in diameter

bull Located at least (3 mm) from fovea or 15 mm) from Optic disc

Group B all remaining discrete tumors confined to retina

All other tumors confined to retina not in group A

Subretinal fluid (without subretinal seeding) lt3 or 3 mm from base of the tumor

Group C local subretinal fluid or seeding

Local subretinal fluid alone gt3 to 6orlt6 mm from the tumor

Viterous seeding or subretinal seeding lt3 or 3 mm from tumor

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

STAGING and CLASSIFICATION

bull ReesendashEllsworth system of classifying retinoblastoma (1964)

bull Predicts the outcome after EBRT

Based on

noof lesion size of lesion

Location of lesion and viterous seeding

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group I Very favorableASolitary tumor less than 4 DD at or behind the

equator B Multiple tumors none larger than 4 DD all at or behind the

equatorGroup II Favorable A Solitary tumor 4ndash10 DD at or behind the equator B Multiple tumors 4ndash10 DD all at or behind the equator

Group III Doubtful A Any lesion anterior to the equator B Solitary tumors larger than 10 DD behind the equator

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group IV Unfavorable

A Multiple tumors some larger than 10 DD

BAny lesion extending anterior to the oraserrata

Group V Very unfavorable

A Massive tumors involving more than half of the retina

BVitreous seeding

RE system DEMERITS

bull Doesnrsquot take into account retinal detachment or subretinal

tumor seeding prognostic factors for vision preservation

bull Small anterior tumors were placed in group 3 or 4 because

they were not usually controlled by ExRT technique in use

at that time but they are quite responsive to modern RT

techniques cryotherapy or RT plaque therapy

bull Vitreous seeding classified as group 5b although local

vitreous seeding can often be treated with RT plaque

International classification of retinoblastoma- 2006

Group A small intraretinal tumors away from the disc and foveola

bull No tumor greater than 3 mm in diameter

bull Located at least (3 mm) from fovea or 15 mm) from Optic disc

Group B all remaining discrete tumors confined to retina

All other tumors confined to retina not in group A

Subretinal fluid (without subretinal seeding) lt3 or 3 mm from base of the tumor

Group C local subretinal fluid or seeding

Local subretinal fluid alone gt3 to 6orlt6 mm from the tumor

Viterous seeding or subretinal seeding lt3 or 3 mm from tumor

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group I Very favorableASolitary tumor less than 4 DD at or behind the

equator B Multiple tumors none larger than 4 DD all at or behind the

equatorGroup II Favorable A Solitary tumor 4ndash10 DD at or behind the equator B Multiple tumors 4ndash10 DD all at or behind the equator

Group III Doubtful A Any lesion anterior to the equator B Solitary tumors larger than 10 DD behind the equator

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group IV Unfavorable

A Multiple tumors some larger than 10 DD

BAny lesion extending anterior to the oraserrata

Group V Very unfavorable

A Massive tumors involving more than half of the retina

BVitreous seeding

RE system DEMERITS

bull Doesnrsquot take into account retinal detachment or subretinal

tumor seeding prognostic factors for vision preservation

bull Small anterior tumors were placed in group 3 or 4 because

they were not usually controlled by ExRT technique in use

at that time but they are quite responsive to modern RT

techniques cryotherapy or RT plaque therapy

bull Vitreous seeding classified as group 5b although local

vitreous seeding can often be treated with RT plaque

International classification of retinoblastoma- 2006

Group A small intraretinal tumors away from the disc and foveola

bull No tumor greater than 3 mm in diameter

bull Located at least (3 mm) from fovea or 15 mm) from Optic disc

Group B all remaining discrete tumors confined to retina

All other tumors confined to retina not in group A

Subretinal fluid (without subretinal seeding) lt3 or 3 mm from base of the tumor

Group C local subretinal fluid or seeding

Local subretinal fluid alone gt3 to 6orlt6 mm from the tumor

Viterous seeding or subretinal seeding lt3 or 3 mm from tumor

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

ReesendashEllsworth system of classifying retinoblastoma (1964)

Group IV Unfavorable

A Multiple tumors some larger than 10 DD

BAny lesion extending anterior to the oraserrata

Group V Very unfavorable

A Massive tumors involving more than half of the retina

BVitreous seeding

RE system DEMERITS

bull Doesnrsquot take into account retinal detachment or subretinal

tumor seeding prognostic factors for vision preservation

bull Small anterior tumors were placed in group 3 or 4 because

they were not usually controlled by ExRT technique in use

at that time but they are quite responsive to modern RT

techniques cryotherapy or RT plaque therapy

bull Vitreous seeding classified as group 5b although local

vitreous seeding can often be treated with RT plaque

International classification of retinoblastoma- 2006

Group A small intraretinal tumors away from the disc and foveola

bull No tumor greater than 3 mm in diameter

bull Located at least (3 mm) from fovea or 15 mm) from Optic disc

Group B all remaining discrete tumors confined to retina

All other tumors confined to retina not in group A

Subretinal fluid (without subretinal seeding) lt3 or 3 mm from base of the tumor

Group C local subretinal fluid or seeding

Local subretinal fluid alone gt3 to 6orlt6 mm from the tumor

Viterous seeding or subretinal seeding lt3 or 3 mm from tumor

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

RE system DEMERITS

bull Doesnrsquot take into account retinal detachment or subretinal

tumor seeding prognostic factors for vision preservation

bull Small anterior tumors were placed in group 3 or 4 because

they were not usually controlled by ExRT technique in use

at that time but they are quite responsive to modern RT

techniques cryotherapy or RT plaque therapy

bull Vitreous seeding classified as group 5b although local

vitreous seeding can often be treated with RT plaque

International classification of retinoblastoma- 2006

Group A small intraretinal tumors away from the disc and foveola

bull No tumor greater than 3 mm in diameter

bull Located at least (3 mm) from fovea or 15 mm) from Optic disc

Group B all remaining discrete tumors confined to retina

All other tumors confined to retina not in group A

Subretinal fluid (without subretinal seeding) lt3 or 3 mm from base of the tumor

Group C local subretinal fluid or seeding

Local subretinal fluid alone gt3 to 6orlt6 mm from the tumor

Viterous seeding or subretinal seeding lt3 or 3 mm from tumor

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

International classification of retinoblastoma- 2006

Group A small intraretinal tumors away from the disc and foveola

bull No tumor greater than 3 mm in diameter

bull Located at least (3 mm) from fovea or 15 mm) from Optic disc

Group B all remaining discrete tumors confined to retina

All other tumors confined to retina not in group A

Subretinal fluid (without subretinal seeding) lt3 or 3 mm from base of the tumor

Group C local subretinal fluid or seeding

Local subretinal fluid alone gt3 to 6orlt6 mm from the tumor

Viterous seeding or subretinal seeding lt3 or 3 mm from tumor

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

Group D diffuse subretinal fluid or seeding bull Subretinal fluid alone gt6 mm from the tumorbull Viterous seeding or subretinal seeding gt3mm from tumor

Group E presence of one or more of these poor prognostic factors More than 23 of the globe filled with tumor Tumor in the anterior segment Tumor in or on the ciliary body Iris neovascularization Neovascular glaucoma Opaque media from hemorrhage Phthisical or prephthisical eye Orbital cellulitis-like presentation

Modified from Shields CL etalinternation classification predicts chemoreduction successopthamology 1132276-22802006

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

International retinoblastoma staging system proposed - 2006

Stage 0 Patient treated conservatively

stage-1 eye eneucleated completely resectedhistologicaly

stage-2 eye eneucleated micoscopic residual tumor

stage-3 regional extension

3a- overt orbital disease

3b- preauricular or cervical lymphnode extension

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

bull stage-4 metastatic diseasebull 4a- heamatogenous metastasis without CNS

involvementbull 1- single lesionbull 2- multiple lesionbull 4b-CNS extension with or without any other site

of regional or metastatic diseasebull 1-prechiasmatic lesionbull 2 CNS massbull 3 leptomeningeal and cerebrospinal fluid disease

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

Goal of treatment

bull Save life (main goal)

bull Preserve useful vision

bull Attention on late functional and craniogeniceffect as sequale to treatment

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

TREATMENT

MODALITIES

Surgery Enucleation exenteration

Focal therapies

Cryotherapy

Laser hyperthermia

Laser photocoagulation

Radioactive plaque applications

External beam radiotherapy

Chemotherapy

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

ENUCLEATIONIndications

bull UL or BL Rb when eye is blind

bull Presence of neovascular glaucoma

bull When disease cannot be controlled by chemo or local treatment

bull Tumor invasion into anterior chamber

bull Direct visulization of an active tumor is obstucted by condition like ndash

hemorrhage corneal opacity or cataract

In co BL Rb

Eye with RD Vitreous hemorrhage glaucoma painful blind eye should

be enucleated and other eye should be treated as per the disease

status

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

Structures removed

bull Gentle removal of intact eye globe without

perforation to avoid seeding of orbit

bull Optic nerve long segment (15mm) for presence of

tumor extension

bull Enucleation is curative in 95 of patient with ul ds

bull Now a days orbital implants are used at mosr centers

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

EXENTERATION

Indications

bull Extensive local tumor breaching the globe- orbital involvement

bull (exenteration in this situation generally is followed by postoperative

radiotherapy and chemotherapy)

bull Recurrence of tumor in the socket after enucleation

Structures removed

bull The globe

bull Extraocular muscles

bull Lids

bull Optic nerve

bull Orbital fat

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

FOCAL THERAPIES

Small tumors( 3-6mm)

Used in combination with chemo

Used as a part of consevative therapy to avoid enucleation and EBRT in ul ds

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

PHOTOCOAGULATION

INDICATIONS

bull Tumors up to 45 mm at the base and up to 25 mm thick not close to the macula or disk with no

evidence of viteros seeds

bull Tumors situated at or posterior to equator of the eye

bull May be used for small tumor recurrences after irradiation

bull Local control of intraocular disease post chemoreduction

TECHNIQUE

Argon laser diode laser or xenon arc photocoagulation are used

bull Based on obliteration of the retinal vessels

bull 2-3 monthly sessions are required

bull LOCAL TUMOR CONTROL 70

Shields JA et alThe expanding role of laser photocoagulation for intraocular tumors The 1993 H Christian

Zweng Memorial Lecture Retina 199414(4)310-22

Complications

Transient serous retinal detachment

bull Retinal traction

bull Retinal hole

bull Visually significant retinal vascular occlusion

CRYOTHERAPY

INDICATIONS

bull Small equatorial and peripheral lesion that lt35 mm at the base and lt2mm in

height

bull Local recurrence

bull Tumor persistence after irradiation

bull In conjunction with chemotherapy

TECHNIQUE

Nitrous oxide probe (-80 C) 1-2 monthly session of triple freeze thaw cycle

SIDE EFFECTS transient serous retinal detachment retinal tear localized preretinal

fibrosis

Transpupillary thermotherapy

PRINCIPLE

bull thermotherapy has synergistic effect with chemotherapy9 ( carboplatin) ndash enhances the platinum

DNA adduct thus ndash antitumor effect

INDICATIONS

bull Small (lt 3mm) tumors posterior to the equator

bull Larger tumors post chemotherapy

TECHNIQUE

bull With diode laser focused heat at sub photocoagulation level- 42C to 60C for 5-20 min to tumor is

applied with relatively sparing of retinal vessels from photocoagulation

bull Local control of 70-80

COMPLICATIONS

bull Retinal traction fibrosis transient serous retinal detachment

RADIOACTIVE PLAQUE APPLICATION

INDICATIONS

1Primary plaque therapy ndash localized RB typically limited to 1-2 foci not involving optic disc or macula

2 In conjunction with chemoreduction therapy- -small or moderate size lesion including those with focal

viterous seeding immediately overlying the tumor

3 Tumors lt1616mm at base and lt8mm in thickness

4 Local failure after other therapy (EBRT chemotherapy laser cryotherapy)

ADVANTAGES (vs EBRT)

bull Better dose localization

bull Lesser risk of cataract

bull Minimal risk of bone hypoplasia

bull Lesser risk of second malignancies

PLAQUE TYPES and features

bull Co60

bull I125 (27 to 35 kev half- life ndash 60 days)- gamma rays

emitter

bull Au198 Ir192Ru106- beta emitter

bull Ir-192- (295-612kevhalflife- 74 days)

bull Ru-106( 35 Mev374 days)

TECHNIQUE

1 Tumor anatomy assessed by USG

2 Incision at limbus

3 Dummy plaque with 2mm margin

4 Retention Sutures placed in sclera

5 Dummy replaced with radioactive

plaque

6 Retention sutures secured

7 Conjunctiva closed

8 Plaque removed after treatment

completion

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

CRYOTHERAPY

INDICATIONS

bull Small equatorial and peripheral lesion that lt35 mm at the base and lt2mm in

height

bull Local recurrence

bull Tumor persistence after irradiation

bull In conjunction with chemotherapy

TECHNIQUE

Nitrous oxide probe (-80 C) 1-2 monthly session of triple freeze thaw cycle

SIDE EFFECTS transient serous retinal detachment retinal tear localized preretinal

fibrosis

Transpupillary thermotherapy

PRINCIPLE

bull thermotherapy has synergistic effect with chemotherapy9 ( carboplatin) ndash enhances the platinum

DNA adduct thus ndash antitumor effect

INDICATIONS

bull Small (lt 3mm) tumors posterior to the equator

bull Larger tumors post chemotherapy

TECHNIQUE

bull With diode laser focused heat at sub photocoagulation level- 42C to 60C for 5-20 min to tumor is

applied with relatively sparing of retinal vessels from photocoagulation

bull Local control of 70-80

COMPLICATIONS

bull Retinal traction fibrosis transient serous retinal detachment

RADIOACTIVE PLAQUE APPLICATION

INDICATIONS

1Primary plaque therapy ndash localized RB typically limited to 1-2 foci not involving optic disc or macula

2 In conjunction with chemoreduction therapy- -small or moderate size lesion including those with focal

viterous seeding immediately overlying the tumor

3 Tumors lt1616mm at base and lt8mm in thickness

4 Local failure after other therapy (EBRT chemotherapy laser cryotherapy)

ADVANTAGES (vs EBRT)

bull Better dose localization

bull Lesser risk of cataract

bull Minimal risk of bone hypoplasia

bull Lesser risk of second malignancies

PLAQUE TYPES and features

bull Co60

bull I125 (27 to 35 kev half- life ndash 60 days)- gamma rays

emitter

bull Au198 Ir192Ru106- beta emitter

bull Ir-192- (295-612kevhalflife- 74 days)

bull Ru-106( 35 Mev374 days)

TECHNIQUE

1 Tumor anatomy assessed by USG

2 Incision at limbus

3 Dummy plaque with 2mm margin

4 Retention Sutures placed in sclera

5 Dummy replaced with radioactive

plaque

6 Retention sutures secured

7 Conjunctiva closed

8 Plaque removed after treatment

completion

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

Transpupillary thermotherapy

PRINCIPLE

bull thermotherapy has synergistic effect with chemotherapy9 ( carboplatin) ndash enhances the platinum

DNA adduct thus ndash antitumor effect

INDICATIONS

bull Small (lt 3mm) tumors posterior to the equator

bull Larger tumors post chemotherapy

TECHNIQUE

bull With diode laser focused heat at sub photocoagulation level- 42C to 60C for 5-20 min to tumor is

applied with relatively sparing of retinal vessels from photocoagulation

bull Local control of 70-80

COMPLICATIONS

bull Retinal traction fibrosis transient serous retinal detachment

RADIOACTIVE PLAQUE APPLICATION

INDICATIONS

1Primary plaque therapy ndash localized RB typically limited to 1-2 foci not involving optic disc or macula

2 In conjunction with chemoreduction therapy- -small or moderate size lesion including those with focal

viterous seeding immediately overlying the tumor

3 Tumors lt1616mm at base and lt8mm in thickness

4 Local failure after other therapy (EBRT chemotherapy laser cryotherapy)

ADVANTAGES (vs EBRT)

bull Better dose localization

bull Lesser risk of cataract

bull Minimal risk of bone hypoplasia

bull Lesser risk of second malignancies

PLAQUE TYPES and features

bull Co60

bull I125 (27 to 35 kev half- life ndash 60 days)- gamma rays

emitter

bull Au198 Ir192Ru106- beta emitter

bull Ir-192- (295-612kevhalflife- 74 days)

bull Ru-106( 35 Mev374 days)

TECHNIQUE

1 Tumor anatomy assessed by USG

2 Incision at limbus

3 Dummy plaque with 2mm margin

4 Retention Sutures placed in sclera

5 Dummy replaced with radioactive

plaque

6 Retention sutures secured

7 Conjunctiva closed

8 Plaque removed after treatment

completion

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

RADIOACTIVE PLAQUE APPLICATION

INDICATIONS

1Primary plaque therapy ndash localized RB typically limited to 1-2 foci not involving optic disc or macula

2 In conjunction with chemoreduction therapy- -small or moderate size lesion including those with focal

viterous seeding immediately overlying the tumor

3 Tumors lt1616mm at base and lt8mm in thickness

4 Local failure after other therapy (EBRT chemotherapy laser cryotherapy)

ADVANTAGES (vs EBRT)

bull Better dose localization

bull Lesser risk of cataract

bull Minimal risk of bone hypoplasia

bull Lesser risk of second malignancies

PLAQUE TYPES and features

bull Co60

bull I125 (27 to 35 kev half- life ndash 60 days)- gamma rays

emitter

bull Au198 Ir192Ru106- beta emitter

bull Ir-192- (295-612kevhalflife- 74 days)

bull Ru-106( 35 Mev374 days)

TECHNIQUE

1 Tumor anatomy assessed by USG

2 Incision at limbus

3 Dummy plaque with 2mm margin

4 Retention Sutures placed in sclera

5 Dummy replaced with radioactive

plaque

6 Retention sutures secured

7 Conjunctiva closed

8 Plaque removed after treatment

completion

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

PLAQUE TYPES and features

bull Co60

bull I125 (27 to 35 kev half- life ndash 60 days)- gamma rays

emitter

bull Au198 Ir192Ru106- beta emitter

bull Ir-192- (295-612kevhalflife- 74 days)

bull Ru-106( 35 Mev374 days)

TECHNIQUE

1 Tumor anatomy assessed by USG

2 Incision at limbus

3 Dummy plaque with 2mm margin

4 Retention Sutures placed in sclera

5 Dummy replaced with radioactive

plaque

6 Retention sutures secured

7 Conjunctiva closed

8 Plaque removed after treatment

completion

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

TECHNIQUE

1 Tumor anatomy assessed by USG

2 Incision at limbus

3 Dummy plaque with 2mm margin

4 Retention Sutures placed in sclera

5 Dummy replaced with radioactive

plaque

6 Retention sutures secured

7 Conjunctiva closed

8 Plaque removed after treatment

completion

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

DOSAGE

bull Target dose -40 Gy to the tumor apex for I-125 at 40-80 cGyhr and 120 Gy at

tumor base ( scleral dose)(Tumor Control = 83 Philadelphia)

bull As adjuvant after chemo 35Gy and 1 month after chemotherapy to reduce

toxicity

COMPLICATIONS

bull Retinopathy ( 5 yr acturial rate= 27)

bull Cataracts(31) (plaque used post EBRT)

bull Maculopathy (25)

bull Papillopathy (26)

bull Glaucoma (11) ( post EBRT)

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

EXTERNAL BEAM RADIOTHERAPY

INDICATIONS

bull When RB is multifocal or close to the macula or optic nerve with preserved vision

bull Large tumors not amenable to focal therapies

bull Vitreous seeding

bull Secondary therapy to salvage chemoreduction and focal therapy failures

bull Tumors involving cut end of optic nerve

bull To palliate or consolidate the systemic therapy of metastatic disease

GOALS

bull Tumoricidal dose to the entire retinal and vitreous since

All retinal cells have a genetic neoplastic potential

Vitreous seeding may have occurred

Tumor may have spread via sub retinal space

bull Opposite eye irradiation be avoided if uninvolved

bull Sparing of lacrimal glands cornea

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

LATERAL BEAM MEGAVOLTAGE TECHNIQUE

Lateral field

Ant border at lateral edge of bony orbit

Posterior border at apex of orbit

Superior border at superior bony ridge

Inferior border at inferior bony ridge

bull Direct lateral field if opposite eye is enucleated

bull If opposite eye present then beam slightly angled posteriorly to avoid exit

radiation to other eye

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

ADVANTAGES lsquoDrsquo shaped field produced after

sheilding pituitary and alveolar processes saves

tooth buds and pituitary

SHORTCOMINGS underdosage of the retina bw

oraserrata anteriorly and equator of the globe to

save the contralateral eye

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

DIRECT ANTERIOR FIELD ( McCormick et al)

Borders

Superior superior orbital margin

Inferior inferior orbital margin

Lateral lateral bony canthus

Medial midline

bull ADVANTAGES Treats entire eye

Saves opposite eye

Easy to set up reproducible

Homogenous dose to entire retina and vitreous

bull DISADVANTAGES Cataract almost inevitable

Lacrimal gland dosage produces impaired tear production

Exit beam through brain

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

PROTON BEAM

ADVANTAGES

bull Superior dose distribution

bull Sparing of other eye because of stopping characteristics

bull In BL cases tissue lying in between two eyes can be saved

bull Lowering the risk of radiation induced malignancies

DISADVANTAGES

bull High bone dose while producing spread out proton beam

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

CONFORMAL RADIATION THERAPY

ADVANTAGES Possible to spare lens while irradiating whole of retina

-Substantially less dose to surrounding soft tissue and bones

DISADVANTAGES

Higher integral dose increases risk of second malignancies

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

ExRT DOSAGE

dose to treat a retinoblastoma with ExRT is

40-45 Gy- 18 Gy to 2 Gy

Dose modification needed when using RT

after chemotherapy for consolidation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

Chemotherapy-

Used in three basic situations1 To locally control the intraocular disease

Chemotherapy is used in neoadjuvant setting to reduce the size of large tumors to a size suitable for eradication by focal therapies (chemoreduction)

2 To produce regression of metastatic disease3 To prevent recurrence or metastases of locally

advanced Rb (Adj CT)Chemo is seldom used as a sole treatment modality

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

Intraocular ( Subconjunctival) CarboplatinIntrathecal Methotrexate cytarabine ( Brain mets trilateral Rb)

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

LATE EFFECTS OF RADIOTHERAPY

1Secondary Malignant Neoplasms

bull Childeren woth heriditary retinoblastoma are 3 fold increased risk as compared to non hereditary

Cumulative incidence of secondary malignancy after 50 years of diagnosis-

bull 36 in hereditary

bull And 57 in sporadic

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

In hereditary group

bull standard incidence -22 times the expected incidence after RT and 7 times without RT

In sporadic cases ndash incidence of second neoplasm (breast cancer) 28 times the normal population

bull Childeren who recived RT in 1 yr of life have higher risk of developing second cancer withinfield of radiation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

bull Osteogenic sarcoma (mc)-75

bull Soft tissue sarcoma and melanoma(25)

bull Epithelial tumors(in late adulthood) ndash lung breast and colon

bull Lipoma

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

bull 2 Bony orbital abnormalites Post RT and post

enucleation significant risks of orbital mal development

and midfacial growth retardation has been observed

bull EBRT (gt35Gy) to lt 6month old children accentuates the

risk

3CataractClinically significant posterior pole cataract

Anterior field techniques -85 lens sparing techniques-28

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

EXTRAOCULAR RETINOBLASTOMA

bull Locoregional including orbital infilterationtumor extending into optic nerve or to cut end of the nerve and lymphatic spread to preauricular lymphnodes

2cns disssemination

3Trilateral retinoblastoma

4 Metastatic retinoblastoma

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

Orbital and locoregionalretinoblastoma

bull Orbital RB occurs as aresult of progression of the tumor via emissary vessels and sclera

bull Treatment ndash systemic chemotherapy and radiotherapy- 60-85 cure rate

bull Pt with macroscopic disease- initial chemo fb surgery (obivates the need of orbital exenteration)---post op chemo and radiation 40-45 Gy (consolidative therapy)

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

bull Pt with isolated optic nerve involvement ndash

bull Systemic chemo with radiation to entire orbit (36 Gy ) and 9-10 Gy boost to chiasma (total 45Gy)

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

CNS disease-

bull Occurs by direct extension by optic nerve

bull Rx- platinum based chemotherapy+ craniospinal irradiation( 234 Gy ndash 36 Gy to neuraxis and a boost to 45 Gy to sites of overt or nodular ds)

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

Trilateral retinoblastoma

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation

CONCLUSIONS

bull Early detection and treatment can save the vision in retinoblastoma

bull With the advent of neoadjuvant chemoradiation treatment goal has now

shifted from enucleation to visual preservation in most of patients

bull Combined approach of Ophthalmologist Radiation Oncologist Pediatric

Oncologist Medical Physicist Pathologist Radiologist Geneticist with

individualization of treatment often leads to favorable outcome in terms

of overall survival and vision preservation