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Gliomas
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OPT
• 3-6% of pediatric intracranial tumors
• 75% in the first decade of life
• 15-20% of CNS astrocytomas
• No gender predominance
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OPT - Classification
• Prechiasmatic
• Exophitic
• Diffuse
•Prechiasmatic
•Chiasmatic-hypothalamic
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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)
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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
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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
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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
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Optic nerve glioma (ONG)
• Proptosis Painless Non pulsatile
• Visual loss• Optic atrophy/ papilledema
• Decreased eye movements
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ONG
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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
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ONG
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ONG
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Chiasmatic-hypothalamic tumors (Ch-H)
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Ch-H
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Ch-H
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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
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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 ?
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Ch-H – Options of treatment
• ObservationStable lesionsNF1
• SurgeryRadical subtotalLimited
Decompression Biopsy
• Chemotherapy• Radiation therapy
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Surgery – Subtotal removal
Hoffman, 1983
Albrighth & Selabassi, 1985
Fletcher et al., 1986
Wisoff, 1990
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Surgery – Subtotal removal
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Surgery – Subtotal removal
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Surgery – Subtotal removal
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Operative morbidity and morbidity
Diabetes insipidus
Hypersomnia
Amaurosis
Precocious puberty
ADH ISS
Death
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Limited surgery + Chemotherapy
6m 1y
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Limited surgery + Chemotherapy
Before (02/1998) After (05/2000))
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Biopsy + Chemotherapy
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Current trends
• Avoid radiation therapy
• Delay radiation therapy
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Radiation therapy
Intelectual impairment Visual impairment Endocrine impairment Moyamoya disease Brain infarction Induced tumors
• Relapse
• Older children
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Chemotherapy + Radiation therapy
19971997 19991999 2000
2002
RT2004
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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
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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.