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Maher Hassounah Consultant Neurosurgeon King Faisal Specialist Hospital & Research Center Riyadh
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Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Jun 10, 2018

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Page 1: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Maher Hassounah

Consultant Neurosurgeon

King Faisal Specialist Hospital & Research Center

Riyadh

Page 2: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious
Page 3: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Optic pathway glioma

Page 4: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Grow without intervention (Sx, chemo or radiation)

Grow despite Intervention

Arrest without intervention 20%

Arrest after intervention

Regression and involution without intervention

Regression and involution after intervention

Dissemination or malignant degeneration

Page 5: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Anterior Pathway

Posterior Pathway

NF 1

NF 1

Age

Page 6: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

15% of NF1 patients

10% of all OPG are NF1-associated

Usually affects optic nerve and chiasm rather than hypothalamus

Precocious puberty

Tend to have better prognosis

Page 7: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Genotype–phenotype correlation in NF1

Mutation in the first third of the gene more likely to develop an optic pathway glioma

Sharif et al. J Med Genet. 2011 Apr

Chromosome 17

Page 8: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Lesion is believed to be of Benign Nature Lesion is believed to be of Benign Course & does not cause

complications No histo-patholgical diagnosis is required

Tumors involving exclusively the optic nerve (not progressing)

Tumors that obviously involve the optic chiasm or tracts (not progressing)

Tumors associated with NF1

After partial resection

Page 9: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

NF 1Craniotomy : 25 September 1996No radiation No Chemo

Seizure disorderL-Thyroxin

Page 10: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Control tumor growth

Restore or improve function

Establish diagnosis

Do no harm

Page 11: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Severe proptosis from large tumors

Decompression of large progressing tumorso To relief IC hypertension

o To relief hydrocephalus

o To relief pressure on surrounding structures

Optic pathway glioma in adults

Histo-pathologic diagnosis (optic glioma v/s other tumor)

Molecular genetic analysis

Page 12: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Y-H Chen and D H GutmannOncogene 33, 2019-2026 (17 April 2014)The molecular and cell biology of pediatric low-grade gliomas

Page 13: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

BRAF fusion resulting from focal duplication at 7q34 (BRAF : KIAA1549 fusion)

o Infratentorial tumors > Suprantentorial tumors o The frequency of this change varies from 50 to 100%

BRAF V600E (point mutation): single amino acid changes (valine to glutamate change at position 600)o Suprantentorial tumors > Infratentorial tumors

Activation of the MAPK/ERK signaling pathway (mitogen-activated protein kinase/extracellular signal-regulated kinase) o identified in approximately 80–90% of PA

Pfister S et al. J Clin Invest 2008 May

Bar et al. J Neuropathol Exp Neurol. 2008 Sep

Page 14: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Jones et al. Cell Mol Life Sci. 2012 Jun

Page 15: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Oncogene-induced senescence in PA

Jones et al. Cell Mol Life Sci. 2012 Jun

Page 16: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Before Cellular Senescence

Senescence-associated β-galactosidase

Mouse Fibroblast

Page 17: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

“Fusion-positive tumors” has favorable prognosis in 70 low grade astrocytoma

No association of “Fusion-positive tumors” with progression-free survival (poor prognosis in < 1 year age, incomplete resection in 93 PA cases)

Hawkins et al. Clin Cancer Res 2011

Cin et al. Acta Neuropathol. 2011

Page 18: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious
Page 19: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious
Page 20: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Upper nasal fibres

Macular fibres

Lower nasal fibres

Anterior clinoid

Pituitary gland

III rd ventricle

Craniopharyngioma

Optic chiasm

Diaphragma sellae

Posterior clinoid

Dorsum sellae

Page 21: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Visual field defects in Pituitary Adenomas

LE RE

HM

CF

AnteriorlyDecussating fibresare most vunerable

Page 22: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Craniopharyngioma

LE RE

HM

CF

The posteriorly crossingfibres are most vunerable

Craniopharyngioma

Page 23: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Meningioma

LE RE

Junctional scotoma

Tuberculum Sellameningioma

Olfactory groove meningioma

Sphenoid ridge meningioma

Page 24: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Ophthalmic ArterySup. Hypophysial Artery

Ant. Cer. Arteries & A. Com. ArteryP.Com. Artery

Blood Supply of the Optic Chiasm

Page 25: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious
Page 26: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Rt. Upper & lowerNasal Fibers

Lt. Upper Nasal & Temporal Fibers

R L

Page 27: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious
Page 28: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious

Int. J. Radiation Oncology Biol. Phys., Vol. 56, No. 3, pp. 807–812, 2003

Page 29: Optic Pathway Glioma Surgical Objectives and Outcome · 15% of NF1 patients 10% of all OPG are NF1-associated Usually affects optic nerve and chiasm rather than hypothalamus Precocious