Syafril Umar Lubis

RETINOBLASTOMA (RB) is the most common primary intraocular malignancy of infancy and childhood

The incidence of RB : 1 : 14000 -20000 LIVE BIRTHS

SPORADICALLY OR INHERITED

Mutation of the RB1gene, the q14band of chromosome 13

RB : present with advance diseases in

developing countriesEye enlargement is a common finding

Family history of RB 45-50% have child with RB

Onset : first year of life in bilateral and family history, between 1 and 3 in sporadic unilateral cases

THE GOAL WITH RETINOBLASTOMATHE GOAL WITH RETINOBLASTOMA

Early detection to maximize the visual outcome

The quality of life of the affected child

Early detection to maximize the visual outcome

The quality of life of the affected child

Leukocoria also known as amaurotic cat’s eye

Dilated pupil

The most common sign

Normal red reflex

Red reflex absent

Red reflex abnormal

Strabismus, or decrease vision.

Less common :o Vitreous hemorrhageso Hyphemao Ocular / periocular inflammationo Glaucomao Proptosiso Hypopyon

Screening for RB should be part of “well baby”

screening for new borns during the first three

months of life

The red reflex

The corneal red reflex / Hirshberg test

Eye examination

IF THE EYE EXAMINATION IS ABNORMAL :

o CT Scano Magnetic Resonance Imaging (MRI)

Can help define the structure abnormalities & reveal any calcium deposit

o USGo USG CAN HELP DEFINE THE HEIGHT & THICKNESS

OF THE TUMOR

o BONE MARROW EXAMINATION /o LUMBAR PUNCTION

o BONE MARROW EXAMINATION /o LUMBAR PUNCTION

TO DETERMINE METASTASES TO BONES OR THE BRAINS

o BLOOD TESTo BLOOD TEST

o GENETIC and/or DNA TESTINGo GENETIC and/or DNA TESTING

The large white mass with

prominent feeder vessels is

located at the macula A smaller

tumor is located in the

inferolateral part of the retina.

A B

I Solitary tumor <4 DD Multiple tumors, none >4 DDII Solitary tumor, 4-10 DD Multiple tumors, 4-10 DDIII Any lesion anterior to equator Solitary tumor > 10 DDIV Multiple tumors, some > 10 DD Any lesion anterior OSV Tumor involving 50% of retina Vitreous seeding

Reese-Ellsworth. DD disc diameter

15

Group Characteristics

A Small tumors, ≤ 3 mm, 3 mm from foveola, 1.5 mm from disc

B Tumor > 3 mm, subretinal fluid < 3 mm from the base of tumor

C Vitreous seeding (C1) ≤ 3 mm,or subretinal seeding (C2) ≤ 3 mm from tumor, or both (C3)

D Vitreous seeding (D1) > 3 mm, or subretinal seeding (D2) > 3 mm from tumor, or both (D3)

E No visual potential, poor prognosis, tumor in anterior segment/ cilliary body/ iris neovascularization/ neovascular glaucoma/ vitreous hemorrhage/ phthisis bulbi/ tumor necrosis/ orbital cellulitis-like presentation/ optic nerve/ extraocular tumor

International Classification

16

Cataract Congenital

Persistent hyperplastic primary vitreous

(PHVP)

Coat’s Disease

Retinopathy of Prematurity (ROP)

Survival rate nearly 95 % in last decade (USA)

Early Diagnosis

Prognosis

Recent Advanced

Management

VARIES FROM COUNTRY TO COUNTRY

The priority :

The life of child

Vision

Minimize complication / side effect of

treatment

TREATMENT

The exact course of treatment ≈ individual case

Chemotherapy

Cryotheraphy

External Beam Radiotheraphy

Enucleation

TREATMENT

EXTERNAL BEAM RADIOTHERAPYModerately advanced unilateral or bilateral RB

Diffuse vitreous seedingFailure of chemotherapy or other modilities

EXTERNAL BEAM RADIOTHERAPYModerately advanced unilateral or bilateral RB

Diffuse vitreous seedingFailure of chemotherapy or other modilities

Enucleation 2nd & 3nd management RBnd & 3nd management RB

Massive unilateral RB, Reese-Ellsworth group V or Massive unilateral RB, Reese-Ellsworth group V or

International clssification groups D&EInternational clssification groups D&EEnucleationEnucleationhigh risk histopatologyhigh risk histopatology chemotherapy chemotherapy

reduce the risk of metastasereduce the risk of metastase

Enucleation 2nd & 3nd management RBnd & 3nd management RB

Massive unilateral RB, Reese-Ellsworth group V or Massive unilateral RB, Reese-Ellsworth group V or

International clssification groups D&EInternational clssification groups D&EEnucleationEnucleationhigh risk histopatologyhigh risk histopatology chemotherapy chemotherapy

reduce the risk of metastasereduce the risk of metastase

The combination of ectoposide phosphate, vincristine sulfate and carboplastine is infused every four week 6 month

Indicated for children whose enucleated globes show features high risk metastasis

The combination of ectoposide phosphate, vincristine sulfate and carboplastine is infused every four week 6 month

Indicated for children whose enucleated globes show features high risk metastasis

Can Retinoblastoma be found early ?

All babies should have general eye

exam at birth – six months

Newborns with family history of this

cancer eye exam a few day after

birth, at six weeks, once every three

months until age two

A blood test to determine if the

mutation is present

Can Retinoblastoma be found early ?

All babies should have general eye

exam at birth – six months

Newborns with family history of this

cancer eye exam a few day after

birth, at six weeks, once every three

months until age two

A blood test to determine if the

mutation is present

THANK YOU

THANK YOU