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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. Astrocytic tumors

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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


Opt classification
OPT - Classification

  • Prechiasmatic

  • Exophitic

  • Diffuse

  • Prechiasmatic

  • Chiasmatic-hypothalamic


Histopathology
Histopathology

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)

Pilomixoid astrocytoma

(Tihan et al, 1999)


OPT

Diagnostic criteria of NF1

National Institutes of Health consensus statement on neurofibromatosis (1987)

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

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

Freckling in the axilary or inguinal region;

Optic glioma;

Two or more Lisch nodules (iris hamartomas);

A distinctive osseous lesion (eg, sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis);

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

Sporadic OPT

  • Chiasm and hypothalamus

  • 10 % NF1

  • Isolated

  • Aggressive

  • IC hypertension

OPT and NF1

  • Orbital optic nerve

  • 50-75% of patients have NF1

  • Multicentric tumors

  • Asymptomatic

  • Indolents

  • Proptosis / visual deficit


Opt and nf11
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
Optic nerve glioma (ONG)

  • Proptosis

    • Painless

    • Non pulsatile

  • Visual loss

  • Optic atrophy/ papilledema

  • Decreased eye movements



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








Ch h tumors problems in management
Ch-H tumors : Problems in management

  • Natural history and course

    • Number of patients

    • Variability of treatment options

    • Lack of prospective of studies

  • Biologic behavior

What is the best treatment ?


Ch h options of treatment
Ch-H – Options of treatment

  • Observation

    • Stable lesions

    • NF1

  • Surgery

    • Radical subtotal

    • Limited

      • Decompression

      • Biopsy

  • Chemotherapy

  • Radiation therapy


Surgery subtotal removal
Surgery – Subtotal removal

Hoffman, 1983

Albrighth & Selabassi, 1985

Fletcher et al., 1986

Wisoff, 1990







Limited surgery chemotherapy1
Limited surgery + Chemotherapy

After (05/2000)

Before (02/1998)



Current trends
Current trends

  • Avoid radiation therapy

  • Delay radiation therapy


Radiation therapy
Radiation therapy

Intelectual impairment

Visual impairment

Endocrine impairment

Moyamoya disease

Brain infarction

Induced tumors

Relapse

Older children


Chemotherapy radiation therapy
Chemotherapy + Radiation therapy

1999

2000

1997

RT

2004

2002


Outcome factors affecting long term survival
Outcome : Factors affecting long-term survival

Increasing age

NF1

Chemotherapy

Radiation therapy

Radiation therapy

69% 10y PFS

Grabenbauer et al., 2000

Chemotherapy

34% 5y PFS

89% 5y OS

61% 5y RT FS

Laithier et al., 2003


Opt conclusions
OPT - Conclusions

  • OPT have an unpredictable course despite histological benignity;

  • Tumors associated with NF1 have a most favorable outcome;

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

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

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

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

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