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Opticpathwayglioma
Opticpathwayglioma
Hypothalamic Glioma
1
D R P R AV E E N
K T R I PAT H I
23 May 2016
DEFINITION
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Epidemiology
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INCIDENCE
1% of all intracranial tumours, 3–5% of paediatric brain tumours
and 6% of all orbital tumours.
Gliomas account for two-thirds of all tumours in the optic nerve.
About 25% of OPGs are in the optic nerve [Optic nerve glioma
(ONG)] and 60% involve the chiasm (Optic chiasm glioma).
Nearly one-third of patients with OPG have neurofibromatosis-1
(NF-1).
The mean age at diagnosis was 8.8 years.
The male:female ratio is nearly equal but pure optic nerve
tumours are commoner in girls.
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OPTIC NERVE TUMORS
4
Optic nerve is actually a
tract of central
nervous system,so
primary tumors of the
optic nerve are more
similar
histopathologically to
brain tumors than to
peripheral nerve tumors.
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PATHOLOGY
5
Macroscopic Appearance
ONG can be purely intraorbital or purely intracranial.
Often starts in one compartment and spreads to the other
through the optic foramen causing a dumb-bell pattern .
The chiasmal variety may diffusely infiltrate the
hypothalamus and the floor of the third ventricle- dubbed
as hypothalamic-chiasmatic glioma.
The non-NF-1 OPG in the chiasma may become a large
mass and show areas of cystic change.
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Gross Pathology
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Path images
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Histology: Low-grade Gliomas
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■ Pilocytic astrocytomas
– WHO grade I circumscribed astrocytomas
– Biphasic pattern
▪ Rosenthal fibersand eosinophilic granular bodies
are characteristic
▪ Loose-textured astrocytes and microcysts
▪ Frequently not cystic in the optic pathway
▪ The cells are positive for glial fibrillary acidic
protein.
▪ A higher microvessel density (as assessed by
immunostaining for factor VIII) is thought to
indicate progression
▪ Adult patients may harbour anaplastic astrocytoma or
glioblastoma multiforme rarely Ganglioglioma 23 May 2016
8 The tumour cells in optic nerve
glioma have elongated or hair-
like
appearance (pilocystic).Hence the
name pilocystic astrocytoma.
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9
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Symptoms at Presentation
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Clinical Signs at Presentation
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■ Decreased visual acuity (33%)- early
■ Visual field deficit (33%)- bitemporal himeanopia – chiasmal
involvement.
■ Optic atrophy (61%)
■ Proptosis
■ Painless axial proptosis is commoner in NF-1 patients with OPG, due to the
frequent location of the tumour in the orbit.
■ Painful proptosis is seen only with large tumours and may be due to
compression of branches of the ophthalmic div.
■ Abnormal extraocular muscle exam (21%)
■ Signs of endocrine abnormalities (21%)
■ Ataxia (12%)
■ Papilledema (9%) 23 May 2016
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Central scotomata
altitudinal field defects
At ophthalmoscopy,
the optic disk may be normal in appearance;
alternatively, edema
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13
(a) The right fundus of a 5-year-old girl with a right optic nerve glioma reveals an optic nerve with
mild edema. Her visual acuity was moderately to severely reduced. (b) The left nerve was normal.
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Diencephalic Syndrome
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The symptoms of hypothalamic dysfunction in OPG are commoner in
non-NF-1 patients.
Emaciation despite normal caloric intake in an alert child is known as
the “diencephalic syndrome of infancy”.
Attributed to the dysfunction of the leptin-ghrelin system.
The somatic growth rate is normal in spite of the emaciation
Normal pituitary hormonal function.
Overactivity and pleasantness
Escalating to euphoria & Initial growth acceleration
Autonomic disturbance
Skin pallor, erratic temperature control, diaphoresis, heat intolerance
Spasmus nutans consists of disconjugate nystagmus, torticollis and
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titubation.
MALIGNANT GLIOMA OF ADULTHOOD
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OCHGs and Neurofibromatosis type 1
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■ Associated with neurofibromatosis
type 1 (NF-1)
– 14-40% of NF-1 patients will
develop OCHGs
■ Traditionally
– Tumors associated with NF-1 are
considered to behave less
aggressively
– Higher incidence of spontaneous
regression
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Imaging Characteristics
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■ General characteristics
– Solid, cystic, or combination
– Classically described as globular/ exophytic
suprasellar mass
■ CT
– Low-density to isodense
– Intense enhancement with contrast
■ MRI
– T1: Low-intensity with marked gadolinium
enhancement- tubular or fusiform nerve in axial
– T2: Hyperintense mass
■ Found to grow postero-superiorly with invagination
of the third ventricle
■ With lateral progression, may involve the Circle of
Willis
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•-(A and B)(CECT) of a 4-
year-old boy without C
neurofibromatosis-1 A B
•Note the non-uniform
enhancement in the suprasellar
mass, which fills the
thirdventricle.
•The boy underwent right
pterional craniotomy and
partial excision of chiasmal
pilocytic astrocytoma followed
bybilateral shunt placement. (C
and D) The CECT scans after E
surgery—note the residual D
tumour. He was given
chemotherapy initially.
Radiation was given after he
completed 7 years of age.
•(E and F) The CECT scans
obtained 5 years after
initialpresentation—note the
tumour shrinkage. The vision
remains stable in the left eye
and the growth is near normal
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22
MRI
(A) Non-contrast
coronal magnetic
resonance (MR).
(B) Contrast
sagittal MR of a
20-year-old
female with
nonneurofibroma
tosis-1
hypothalamic-
chiasmal glioma.
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23
Fat-suppressed T1 axial
MRI, without (A) and
with (B) gadolinium
showing bulbous
enlargement of the right
optic nerve (arrow),
consistent with an optic
nerve glioma.
The right eye is
proptotic. Enhancement
extended into the optic
canal (not shown), but
there was no
enhancement or
enlargement of the
chiasm.
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24
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Differenial diagnosis
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Pituitary adenoma
Craniopharyngioma
Germ cell tumour
Hypothalamic hamartoma
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Natural History
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Problems in the Literature: Sources
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■ The existing literature is derived from a wide range of
sources
– NF-1 clinics predominately see indolent tumors
– Neuro-oncologists and radiation oncologists see mostly
those children with progressive disease
– Neuro-ophthalmologists primarily see intra-orbital optic
nerve tumors
– Neurosurgeons
▪ Typically, patients present to neurosurgical service following
the first diagnostic CT or MRI
▪ Broad spectrum of disease prior to the evolution of the natural
course
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Problems in the Literature: The Importance of
Anatomic Location
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■ Tumors grouped together regardless of anatomic location
– Tumors of the optic apparatus
– Optic pathway gliomas
Survival
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The Dodge Classification Systems for Optic
Pathway30
Gliomas
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Observation
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Spontaneous Regression
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Surgery
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In contrast to the cerebellar juvenile pilocytic astrocytoma, surgery
plays only a limited role in OPG
Radical surgery has not been proved to increase the length or
quality of survival.
Indications for surgery in optic pathway glioma
Biopsy for tumours with atypical imaging findings
Mass reduction by cyst drainage and tumour decompression for relieving
raised pressure symptoms or neighbourhood compression syndromes
Disfiguring or painful proptosis
Total excision for orbital optic nerve tumours with total visual loss, so as to
prevent intracranial extension and to provide cure without adjuvant therapies
Excision of exophytic masses (mainly chiasmal)
Ventricular shunt placement for hydrocephalus
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intraorbital tumours
35
• Subfrontal
– Lateral (pterional)
– Anterior
• Transventricular
– Interhemispheric transcallosal
– Anterior interhemispheric, trans-lamina terminalis
– Endoscopic transventricular
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37
Enucleation
specimen with
optic nerve glioma
from a
patient with NF1.
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Chemotherapy
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Chemotherapy
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■ Temozolomide (Temodar)
– Gururangan ’07: N=30, 54% disease stabilization,
5yr PFS = 30%
– Nicholson ’07: N=21, 57% disease stabilization
■ Avastin/CPT11
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Radiotherapy
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■ Significant toxicity
– Vision worse in 7-14%
– Endocrine dysfunction: Panhypopituitarism, GH deficiency
– Cerebrovascular Disease
▪ Moya Moya in 3/5 (60%) NF+, 2/23 (9%) NF- (Kestle ’93 )
▪ Vascular disease in 11/37 (30%) NF+, 2/32 (6%) NF- (Grill ’99 )
■ Secondary tumors
■ Neurocognitive Deficits
▪ Cappelli ’98: 18/51 (35%), 12 with mental retardation, all had
received radiation
▪ Lacaze ’03: Mean IQ 19 points lower in the patients receiving
radiation
▪ Sutton ’95: N=33
– 43% in special education (all received RT, mean age 5.7 yrs, only
3 > 6yo)
– 57% in regular school (4 no RT) (12 RT, mean age 11yrs, 4 were
5-8yo)
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Radiotherapy
45
The recognition of endocrine and cognitive side effects of
radiotherapy in young children has made chemotherapy the choice
in this age.
69% children below 10 years developed hypothalamic- pituitary
endocrine deficiency after radiation
Initial modality in older children >10 year and adults.
The standard prescription is 45–50 Gy in 2 Gy daily fractions to the
tumour and the surrounding 0.5–1 cm margin.
Progression-free survival probabilities after radiotherapy were 82% at 5 years and
77% at 10 years after radiotherapy for OPG
The visual acuity improved in 36%, remained stable in 52% and deteriorated in
12% after radiotherapy in a German series of 25 patients.
The risk of developing a second (usually malignant and lethal) tumour after
radiotherapy is real and is more so for NF-1 patients.
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Radiotherapy
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Classification of Optic Glioma by Factors
Influencing Prognosis
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CONCLUSION
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