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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.