Gliomas

OPT

• 3-6% of pediatric intracranial tumors

• 75% in the first decade of life

• 15-20% of CNS astrocytomas

• No gender predominance

OPT - Classification

• Prechiasmatic

• Exophitic

• Diffuse

•Prechiasmatic

•Chiasmatic-hypothalamic

Histopathology

Pilomixoid astrocytoma(Tihan et al, 1999)

Astrocytic tumors

1.1.1 Astrocytoma - Fibrilary, protoplasmátic, gemystocític

1.1.2 Anaplastic astrocytoma (malignant)

GCGB, Gliosarcoma

1.1.3 Multiform gliblastomas

1.1.4 Pilocytic astrocitoma

1.1.5 Pleomorphic xantoastrocitoma

1.1.6 GCSA(Tuberous sclerosis)

OPT

Diagnostic criteria of NF1National Institutes of Health consensus statement on

neurofibromatosis (1987)

1. Six or more café-au-lait macules larger than 5mm in greatest diameter in prepubertal individuals and larger than 15 mm in postpubertal individuals;

2. Two or more neurofibromas of any type or one plexiform neurofibroma;

3. Freckling in the axilary or inguinal region;

4. Optic glioma;5. Two or more Lisch nodules (iris

hamartomas);6. A distinctive osseous lesion (eg,

sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis);

7. A first degree relative (parent, sibling, or offspring) with neurofibromatosis 1 according the above criteria.

(Two or more criteria are needed for a diagnosis of NF1)

• Neurofibromatosis 1

• Sporadic

OPT and NF1

OPT and NF1

• Orbital optic nerve

• 50-75% of patients have

NF1

• Multicentric tumors

• Asymptomatic

• Indolents

• Proptosis / visual deficit

Sporadic OPT

• Chiasm and hypothalamus

• 10 % NF1

• Isolated

• Aggressive

• IC hypertension

OPT and NF1

“OPG in patients without NF differs significantly from NF-OPG in both imaging features and prognosis. Non NF-OPG and NF-OPG are apparently distinct entities, each warranting a specific diagnostic, clinical and therapeutic

approach.” Kornreich L et al., 2001

Optic nerve glioma (ONG)

• Proptosis Painless Non pulsatile

• Visual loss• Optic atrophy/ papilledema

• Decreased eye movements

ONG

ONG

Surgical indications

• Loss of unilateral vision (blindness)

• Progressive visual loss

• Exophtalmos

Surgical contraindications

• Partially preserved vision

• Moderate proptosis

• Stable lesions

• Contralateral extension or

chiasmal envolvement

ONG

ONG

Chiasmatic-hypothalamic tumors (Ch-H)

Ch-H

Ch-H

Signs and symptoms

Intracranial hypertension Decreased visual acuity Visual fields deffects Precocious puberty Macrocrania Russel syndrome Seizures Motor signs Nistagmus Diabetes insipidus Mental retardation Hypersomnia

Chiasmatic

Hypothalamic

Ch-H tumors : Problems in management

• Natural history and courseNumber of patients

Variability of treatment options

Lack of prospective of studies

• Biologic behavior

What is the best treatment ?

Ch-H – Options of treatment

• ObservationStable lesionsNF1

• SurgeryRadical subtotalLimited

Decompression Biopsy

• Chemotherapy• Radiation therapy

Surgery – Subtotal removal

Hoffman, 1983

Albrighth & Selabassi, 1985

Fletcher et al., 1986

Wisoff, 1990

Surgery – Subtotal removal

Surgery – Subtotal removal

Surgery – Subtotal removal

Operative morbidity and morbidity

Diabetes insipidus

Hypersomnia

Amaurosis

Precocious puberty

ADH ISS

Death

Limited surgery + Chemotherapy

6m 1y

Limited surgery + Chemotherapy

Before (02/1998) After (05/2000))

Biopsy + Chemotherapy

Current trends

• Avoid radiation therapy

• Delay radiation therapy

Radiation therapy

Intelectual impairment Visual impairment Endocrine impairment Moyamoya disease Brain infarction Induced tumors

• Relapse

• Older children

Chemotherapy + Radiation therapy

19971997 19991999 2000

2002

RT2004

Outcome : Factors affecting long-term survival

• Increasing age

• NF1

• Chemotherapy

• Radiation therapy

Radiation therapy69% 10y PFS

Grabenbauer et al., 2000

Chemotherapy34% 5y PFS89% 5y OS61% 5y RT FS

Laithier et al., 2003

OPT - Conclusions

1. OPT have an unpredictable course despite histological benignity;

2. Tumors associated with NF1 have a most favorable outcome;

3. Age is the most important prognostic factor and younger children have the worst prognosis;

4. Unilateral ONGs associated with blindness and proptosis are best treated with radical resection;

5. Subtotal radical surgery of Ch-H tumors is feasible but is associated with a high morbidity;

6. Chemotherapy seems to be a reasonable alternative as first line treatment of Ch-H tumors;

7. Prospective, randomized, multicentric studies are needed in order to know the natural history and define the best treatment of OPT.