Optic nerve tumors ppt

OPTIC NERVE
TUMORS
Moderator: Dr. S.C. Bubanale
Presenter: Dr. Arushi Prakash
1Department of Ophthalmology, JNMC
29th October ’14

Contents
# Optic Nerve Anatomy
Tumors of theOptic Nerve
# Optic nerve Gliomas
# Primary Optic Nerve Sheath Meningioma
# Secondary Optic Nerve Sheath Meningioma
# Optic Nerve Melanocytoma
# MalignantAstrocytoma
# Astrocytic Hamartoma
# LymphoreticularTumors
# Metastatic Tumors
Wednesday, October 29, 2014

Optic nerve :
The optic nerve being a portion of central nervous
system is a tract, and not really a nerve.
However, as a convention the
1.2 million axons that derive
from the retinal ganglion cells
carry the name optic nerve,
until they partially decussate
at the optic chiasm.
Wednesday, October 29, 2014 Department of Ophthalmology, JNMC 3

• Optic nerve is about 50 mm long and
extends from the eye to optic chiasm.
• it carries about 1.2 million axons that
derive from the retinal ganglion cells.
• It consists of 4 portions :
A)intraocular portion: optic disc- 1 mm in antero
posterior direction.
B) intra orbital portion : about 25 mm long.
C) intra canalicular portion with in the optic canal :
about 9mm long.
D)intracranial portion : about 16mm long.

Constituent elements of optic nerve :
• Myelinated axons retinal ganglion cells.
• Oligidendrocytes : These are specialized glial cells
that provide membranes for axonal myelination.
• Microglia : These immunocompetant cells play a role
in protection of optic nerve from infection and also
suggested that these cells probably play a role in
apoptosis of retinal ganglion cells.

Astrocytes:
-Astrocytes have extensive neurofibrillary processes
that spread among nerve fibres. They form a part of
blood brain barrier, and play a role in the nutritional
and structural support of axons.
-when axons are lost because of optic atrophy ,
astrocytes move and proliferate to fill the empty
spaces.

OPTIC NERVE TUMORS
• 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.

Optic Nerve Glioma

• Pilocytic hamartomas of the anterior
visual pathways.
• More posterior gliomas are generally
more aggressive variants.
• Comprise ∼1% of all intracranial tumors
• About half are confined to the orbit; the remainder
have intracranial extension
• manifest themselves early most commonly in the
first decade of life (70% detected during the first
decade, 90% by second)
• no definite sex predilection

• Roughly 29% of optic gliomas are associated with
NF1
• Among patients with NF1, optic gliomas have been
estimated to occur in 14.7-17.4%
• Isolated (without association to NF1) optic nerve
gliomas are usually unilateral
• Bilateral involvement is believed to be
pathognomonic of NF1

Enucleation
specimen with
optic nerve
glioma from a
patient with NF1.

Pathogenesis
• The NF1 gene product, neurofibromin, has a GTPase-
activating protein domain that interacts with the Ras
protein, which is crucial in regulating signal
transduction and cell proliferation and
differentiation.
• Patients with NF1 are at high risk for developing a
number of different types of tumors because of this.
• At this point, the exact mechanism of action of
development of these tumors is unknown.

• The most common presenting findings are
• proptosis (94%).
• visual loss (87.5%).
• optic disc pallor (59%).
disc edema (35%).
• and strabismus (27%).
• Patients infrequently
present with asymptomatic
isolated optic atrophy.
• An RAPD is usually present in unilateral or
asymmetric cases
Clinical Features

• typical optic nerve- related visual field defect (if the
patient is cooperative enough for visual field testing).
• Retinochoroidal collaterals may be present on the
affected disc
• Central retinal vein occlusion or venous stasis
retinopathy may occur.
• Large tumors may cause obstructive hydrocephalus
with elevated ICP, headache, and papilledema.

Face photo of a 5-year-old girl who developed noticeable
proptosis OD and found to harbor an optic nerve glioma.
(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.

• In rare cases, the optic glioma can resolve
spontaneously
• Survival is typically reduced when hydrocephalus is
present.

Diagnosis is confirmed by
neuroradiologic findings
• Fusiform or globular enlargement of the optic nerve
• Thickening of both nerve and sheath by tumor
• Kinking or buckling of the optic nerve
• Regions of low intensity within the nerve (cystic
spaces)
• Smooth sheath margins (no extradural extension)
• No calcification or hyperostosis on CT scan
• Isointense or mildly hypointense to brain on T1 -
weighted MRI scan
• Hyperintense on T2-weighted MRI scan

biopsy of the mass is generally not
required :
 advent of high -resolution neuroimaging has raised
diagnostic accuracy
 biopsy of sheath alone may be inaccurate with
reactive meningeal hyperplasia in gliomas falsely
suggesting meningioma
 biopsy of the optic nerve substance may produce
additional visual loss
 the histopathologic appearance of the tumor is not
necessarily predictive of biological behavior

Pathology
• When confined to the orbital portion of the optic
nerve, a glioma often appears as a fusiform swelling
• Histologically, an optic nerve glioma is generally
characterized as a juvenile pilocytic astrocytoma,
characterized by cells with prominent eosinophilic
processes called Rosenthal's fibers. Mixed gliomas
and oligodendrogliomas have been reported as well.
• The tumor may infiltrate the normal optic nerve
substance or be confined primarily to the
subarachnoid space.

Department of Ophthalmology, JNMC 21
The tumour cells in optic nerve
glioma have elongated or hair-
like
appearance (pilocystic).Hence
the name pilocystic
astrocytoma.
Rosenthal fibres in optic nerve
glioma. These are degenerative
eosinophilic substances found within
the cytoplasm of astrocytes.
There are characteristic but not
diagnostic of optic nerve glioma

• The latter type is more commonly found in patients
with neurofibromatosis type 1.
• Rarely, there is exophytic extension of intracranial
optic-chiasmal gliomas.
• The overall histologic appearance is Benign, with a
paucity of cellular atypia, mitosis, or tumor necrosis.
Often, microcystoid extracellular spaces containing
acid mucopolysaccharide produced by astrocytes are
encountered. Such spaces may constitute a major
portion of the optic nerve mass.

Natural history
• Variable
• patients with the diagnosis of NF1 tend to
fare better with respect to growth and visual
prognosis.
• Most tumors grow slowly or have self-limited
growth.
• some tumors are more aggressive, resulting in a
rapid increase in ipsilateral proptosis and visual loss.
o (more apt to occur in patients who do not have NF1)

Management
• There is no universally accepted
management for optic nerve glioma.
• Observation is indicated for patients with relatively
good vision and stable radiographic appearance.
• Most patients show stability or very slow progression
over years and sometimes show
spontaneous regression.
• Chemotherapy is emerging as the first-line treatment
when visual loss is severe at presentation or
there is evidence of progression.

• Combination carboplatin and vincristine
(Oncovin) is the most accepted regimen.
• Radiotherapy is controversial because of the
inconclusive results and potential complications,
including panhypopituitarism and mental
retardation.
• stereotactic fractionated radiotherapy for optic nerve
gliomas have been found to show good results

• Surgical excision may be indicated in
patients with severe visual loss in association
with disfiguring proptosis.
• Surgery has been advocated to prevent advancement
into the chiasm; however, extension to the chiasm is
rare.

MALIGNANT GLIOMA OF
ADULTHOOD
• Since the advent of CT and MRI, the exceedingly rare
malignant gliomas of the optic nerve and chiasm are
more frequently being diagnosed before death.
• Almost invariably, patients present with rapidly
progressive unilateral or bilateral visual loss.
• Eye pain or headache may accompany the visual loss
and blindness occurs within weeks of onset.

 Central scotomata
 altitudinal field defects
 and hemianopias have all been reported.
• At ophthalmoscopy,
 the optic disk may be normal in appearance;
 alternatively, edema
 or atrophy of the optic disk may be present.

• Significant enhancement of the optic nerve after
gadolinium infusion may indicate the presence of
optic nerve glioma even before marked expansion of
the anterior visual pathway
• Even with aggressive radiation therapy prolonging
survival, there is invariably rapid spread of tumor
intracranially, and death occurs within month.
• Histologically, the tumor may be characterized as
either astrocytoma or
glioblastoma multiforme.
Cells spread in the subpial
space, resulting in
progressive vascular
occlusion and
demyelination.Wednesday, October 29, 2014 Department of Ophthalmology, JNMC 29

# Key Features
• Associated withpainless and slowly progressive vision
loss
• Diagnosis is typically in thefirst decade of lifealthough
they may occur at any age
• Whenassociated with neurofibromatosis type 1, a
familialpreponderance may be seen
• Patientswithand withoutneurofibromatosis type 1
who harbor optic nerve gliomas should be followed
conservatively for sometimeto determine rate of
growth

Optic nerve sheath
meningioma

INTRODUCTION
• Optic nerve sheath meningiomas (ONSM) are
separated into two types: primary and secondary.
The primary variety arises from the cap cells of the
intraorbital or intracanalicular optic nerve sheath
while the secondary form arises intracranially in the
region of the sphenoid wing, tuberculum sella, or
olfactory groove .
Saturday, August 22, 2015 Department of Ophthalmology, JNMC 32

PRIMARY OPTIC NERVE SHEATH
MENINGIOMA
• Arises from proliferations of the meningoepithelial
cells lining the sheath of the intraorbital or
intracanalicular optic nerve
• Although these tumors are uncommon ( 1%-2% of all
meningiomas), they account for one third of primary
optic nerve tumors.
• Usually detected in adults age 40-50
• Women affected 3 times more than men

• 4%-7% of optic nerve sheath meningiomas occur in
children.
• May occur more frequently in patients
with neurofibromatosis type 2
and may be bilateral.
• Bilateral tumors, albeit rare, tend to
present at an earlier age

Clinical Features
• Patients may present with the classic diagnostic
triad:
painless, slowly progressive
monocular visual loss
optic atrophy
optociliary shunt
vessels

Clinical Features
• There often is little if any proptosis.
• Roughly 50% will present with disk edema while the
other half will demonstrate pallor.
• Some patients will also show choroidal folds due to
compression behind the globe.
• Optociliary vein collaterals in the presence of vision
loss and optic disk pallor should suggest the
diagnosis of an ONSM

Clinical Features
• Visual fields typically show central and
paracentral scotomata as well as
altitudinal defects, which progress over time
• Despite the typical progression of reduction in visual
function, some patients maintain stable acuity for
years, or up to 30% may even improve.

(a) Fundus of a man with slowly progressive vision loss OD and neuro-imaging
consistent with an optic nerve sheath meningioma of the right optic nerve. The
optic disk is mild elevated and there are several cilioretinal shunt vessels of
the surface of the nerve substance. (b) The left nerve is normal.

optic disc atrophy, with optociliary shunt
vessels (retinochoroidal collaterals visible
at the 8 and 12 o'clock positions)

Neuroradiologic Features of
Optic Nerve Sheath
Meningioma
• Diffuse tubular enlargement of the optic nerve
• Sheath thickening and enhancement, with relative
sparing of optic nerve substance ("tram track“ or "
railroad track" signs)
• Apical expansion of the tumor
• Extradural tumor extension
• Calcification of the nerve sheath on CT scan

• Adjacent bony hyperostosis on CT scan
• Isointense or mildly hyperintense to brain on Tl- and
T2-weighted MRI scan
• Prominent contrast enhancement on CT and MRI
scan

Axial MRI through the
orbit with gadolinium
and fat suppression in a
35-year-old woman
demonstrating an
enhancing mass along
the right optic nerve.
There is also
depression of the
posterior globe on the
right and the right optic
nerve is visible
protruding into the
vitreous due to disk
edema.

Axial CT through the orbits of a 47-year-old
man complaining of transient visual
obscurations in the right eye. The scan
shows a hyperdense, calcified lesion over
the optic nerve, but the most anterior part
demonstrates the typical tram-tracking that
is seen with an optic nerve sheath
meningioma.
Axial CT through the orbits demonstrating
a bright, calcified lesion of the optic nerve
sheath on the left side. The lesion extends
most of the length of the orbital optic nerve
to the optic canal.

There is a cystic lesion of the optic nerve
on the right on this T1-weighted MRI scan
with gadolinium and fat suppression. The
mass was revealed pathologically to be an
ONSM. The optic disk is protruding into the
vitreous due to edema from the tumor.

CT scan reveals "tramtrack sign"; diffuse
enlargement of the right in traorbital optic
nerve extending anteriorly to the
globe, with enhancement of the optic nerve
sheath

Ring sign" in meningioma. Coronal
orbital MRI scan shows similar sheath
enhancement surrounding relatively
normal, darker optic nerve on the right.

Pathology
• Meningiomas probably arise from cap cells that line
the outer arachnoidal surface.
• Two characteristic features- whorls and psammoma
bodies.
• Some meningiomas, called fibroblastic, consist of
elongated cells, suggestive of fibroblasts.
• Syncytial tumors have indistinct cell borders, and
those with prominent vessels are called angioblastic.

characteristic whorls and psammoma bodies of an optic nerve meningioma

Possible locations of intraorbital meningiomas.
Tumor cells may arise from the planum sphenoidale(1)
or from the optic canal (2) and migrate into the subdural space of the intraorbital optic
nerve. Tumors may also arise de novo from within the optic nerve sheaths and remain
intradural (3) or become exophytic masses (4).
Atopic nests of arachnoidal cells within the orbit (5 and 6) may also give rise to
meningiomas.

• characteristic neuro-imaging leave exact diagnosis
rarely in doubt and biopsy can be deferred.
• due to the mimicry exhibited by metastatic breast
tumors, biopsy in patients with an optic nerve sheath
mass and a history of breast carcinoma.
• Breast tumors may also metastasize to an ONSM.
• Arachnoid cysts and sarcoidosis of the optic nerve
sheath should also be considered

• Left alone, most unilateral ONSMs slowly but
inexorably progress to produce blindness of the
involved eye. However, this is not always the case.
Some patients maintain stable vision for years .
• Intraorbital and intracanalicular meningiomas
extend intracranially only rarely and are not fatal.
• In contrast, tumors that begin intracranially often
extend to involve the chiasm and other structures
adjacent to the optic nerve and may lead to
blindness of the other eye.

Radiation therapy
• may also change the clinical appearance of the
fundus.
• As the tumor shrinks, the optociliary venous
collaterals may regress.
• several reports of radiation retinopathy as well as
iritis, dry eye, or orbital pain.

Surgery
• not indicated due to the high likelihood of inducing
blindness.
• (unavoidable dissection of the pial vascular supply of
the optic nerve)
• should be reserved for those patients with eyes that
are already blind or intracranial extension feared to
extend to adjacent critical structures.

• Recently, several series have been published
documenting treatment outcomes with stereotactic
fractionated radiotherapy.
• indicated that some patients will have improvements
in acuity, but more commonly enjoy improvement in
visual field.
• A greater number of patients develop stability in
their visual acuity and visual fields while only a small
number worsen.

Introduction
• Secondary optic nerve meningiomas result
from meningeal tumors that arise inside the
intracranial vault near the sphenoid wing,
tuberculum sella, and inferior frontal lobes.
They cause vision loss typically due to direct
compression of the optic nerve

Epidemiology
• It is very difficult to determine the epidemiology of
these tumors.
• Most likely, these tumors are predominantly seen in
middle-aged and older women.

Pathogenesis
• Recent advances in genetics have allowed
researchers to investigate the frequency of
mutations that arise in these tumors.
• Mutations have been found on chromosome 22, the
chromosome linked with neurofibromatosis type 2.
• Up to 60% of sporadic meningiomas arise from
inactivation of the NF2 tumor suppressor gene,
leaving 40% caused by unknown factors.
• These deletions do not appear to be more frequent
in more aggressive meningiomas.

• The non-NF2 meningiomas are more often of the
meningothelial subtype and are located
preferentially in the anterior skull base.
• Loss of heterozygosity of 18p may occur in a small
number of sporadic meningiomas

• There are likely hormonal influences regarding the
growth of meningiomas.
• The evidence that implicates gender-specific
hormones in the pathogenesis of meningiomas
emanates from data showing increased growth of
meningiomas during pregnancy and change in size
during menses.
• Estrogen receptor expression is lost or reduced in
more benignly growing meningiomas,
• whereas loss of progesterone receptor expression is
an indicator of early recurrence.The finding of
progesterone receptors in only a few meningioma
cells is a favorable prognostic indicator for prolonged
disease-free survival.

• Human epidermal growth factor receptor type 2
(HER2) represents a well-known prognostic factor in
various tumors such as breast carcinomas.
• rate of tumor recurrence shown to be significantly
higher in meningiomas with HER2 over expression
than in HER2-negative meningiomas

Clinical Presentation
• Sphenoid wing meningiomas typically are associated
with much more severe proptosis than primary
ONSM.
• Tuberculum sella meningiomas may also be
associated with vision loss and cranial neuropathies
causing diplopia.
• The clinical course for these tumors is slow. Rarely,
patients who have undergone resection of a
sphenoid wing meningioma may have intermittent
reversal of postoperative proptosis.

Face shot of a woman complaining of proptosis of the left eye. Neuro-
imaging revealed a left sided sphenoid wing meningioma. The left eye
shows lid retraction and lagophthalmos with protrusion of the left globe. (b)
Picture taken from above the patient demonstrating the degree of proptosis
visually.

Radiologic Corelates
• MRI is the imaging modality of choice in these
tumors.
• CT may show characteristic calcification within the
mass on noncontrast imaging.
• MRI imaging with gadolinium will show more detail
regarding a dural tail or other features, akin to a
tumor of the dural sheath
• The tumors typically enhance homogeneously.
• Coronal sections through the cavernous sinus using
T1-weighted images with gadolinium and fat
suppression are ideal for identifying the degree of
tumor spread.

Coronal T1-weighted MRI scan of the brain with gadolinium in a 48-year-old woman
with vision loss in the left eye. The scan demonstrates a large left sphenoid wing
meningioma and there are visible meningeal attachments laterally under the left
temporal lobe as well as medially through the cavernous sinus. There is midline shift of
the brain and hydrocephalus is noted in the left lateral ventricle in the temporal lobe.

Axial T1-weighted MRI scan of
the brain with gadolinium, but
without fat suppression in a 21-
year-old woman with left sided
vision loss. There is a large left
sphenoid wing meningioma that
extends into the left cavernous
sinus. There is also intraorbital
tumor, but due to the lack of fat
suppression on the scan, it is
difficult to tell what the extent of it
is.

Coronal T1-weighted MRI
scan of the brain with
gadolinium in a 54-year-old
woman complaining of
vision loss OD only. She
had frontal lobe findings
and a flat affect. There is a
very large falcine
meningioma that likely
arose from the right
olfactory groove although
this is difficult to prove on
this imaging. There is
midline shift and direct
compression on brain
tissue.

DIFFERENTIAL DIAGNOSIS
• Tumors in region of the sella may be difficult to
differentiate from one another.
• Some masses originally thought to be meningiomas
turn out to be pituitary tumors at pathological
examination, and vice versa.
• Both of these tumors are benign and typically show
fairly homogeneous enhancement,
• whereas more malignant tumors may show cystic
changes and other features suggestive of a more
aggressive character.
• Rarely, a suspected meningioma may be
found to actually be Rosai–Dorfman disease.

TREATMENT
• Since these are slow growing tumors,
conservative management until symptoms
appear.
• visual fields, and regular MRI scans with gadolinium
enhancement are appropriate for patients who have
good vision and no evidence of intracranial or
intracanalicular extension of tumor.
• appropriate to follow a small amount of intracranial
extension with serial MRI

• Craniotomy reserved for patients with
documented progressive intracranial extension of
tumor.
• A standard pterional craniotomy using microsurgical
technique appears to provide the necessary
exposure required for aggressive resection and with
minimal complications.
• Radiation therapy may be coupled with conservative
resection, and shows similar outcomes when
compared to aggressive resection.

• Aggressive resection has the benefit of
eliminating the risk of future complications of
radiation toxicity, however.
• Complete surgical resection is often not possible in
cases of sphenoid wing meningiomas.
• This is often due to invasion of the surrounding
structures such as the cavernous sinus and orbit
• More of these cases require concurrent treatment
with radiation therapy and are associated with tumor
regrowth.

* Olson and colleagues
• Given the histopathological evidence of the
presence of hormone receptors on meningioma
cells, the effect of drugs targeting these receptors
has been studied.
• In in vitro studies by, meningioma cells were
inhibited by mifepristone (RU 486), estradiol-17β,
and progesterone.
• Tamoxifen stimulated growth of meningioma cells in
vitro.

#Key Features
• Associated with painless and slowly progressive vision loss
• Average age of onset in the fifth decade of life
• Minimal proptosis, if any
• Optociliary vein collateralsmay be present on the optic disk
• Neuroimaging is the mainstayfor diagnosis in a typical case
• Radiation istreatment of choice in an eye with documented
progressive vision loss

Melanocytoma

• Melanocytomas are benign melanocytic tumors that
occur typically on the surface of the optic disk, in the
anterior uveal tract, or, more rarely, in the choroid,
although they have been reported to occur in the
sclera or conjunctiva.
• Also termed ‘magnocellular naevus’, which is also
appropriate. This term is concordant with the
histopathologic appearance of these lesions
which are composed of large, regular cells.

Melanocytoma of Disc
• Relatively rare tumors with unknown
prevalence
• Mean age at diagnosis 40-50 years
• Slight female predominance
• Generally unilateral
• Left and right side affected with
similar frequency

Clinical Presentation
• The clinical presentation of melanocytomas
depends on their size and extension.
• may be confined to the optic disk or
• may infiltrate the adjacent choroid and optic disk
without prolapsing through the retina
Subretinal melanocytoma
situated on the optic disk
and adjacent choroid. (a)
Ophthalmoscopy. (b)
Fluorescein angiography,
early phase

• May infiltrate
the retina,
thereby
resulting in
the classical
appearance of
a filamentous
border
Large melanocytoma situated on the optic
disk and choroid and invading the nerve
fiber layer.

• they can also induce papilledema and
may invade the optic nerve.
Melanocytoma of the optic disk associated with papilledema. (a) Ophthalmoscopy.
(b) Fluorescein angiography, arteriovenous phase

• lesions are invariably dark brown or black and,
apart from rare exceptions, coloration is
homogeneous.
• Size variable, generally do not exceed 2–3 mm in
greatest dimension.
• Adjacent choroid involved in 47-86% of cases
• retina is involved in 30-67% of cases.
• That portion of the optic disk that is spared by the
tumor may appear physiologic or, in ∼25% of the
cases, may reveal papilledema on ophthalmoscopic
examination

• Another 25% of the cases will show
papilledema only on fluorescein angiography
• The tumor can, in rare cases, be accompanied
by a serous retinal detachment, retinal edema,
lipid deposits, or intraretinal haemorrhage and, more
rarely, by polypoidal choroidal vasculopathy
• The vitreous is generally clear, but pigment
dispersion secondary to tumor necrosis may be
observed and may, in some cases, extend to
the anterior chamber

Melanocytoma of the optic disk associated
with marked pigment dispersion in the
vitreous.

• About 75% of the patients are asymptomatic, and the tumor
is discovered incidentally.
• Blurred vision, floaters, flashes, metamorphopsias, or
micropsias are the symptoms most frequently reported in the
other cases.
• Visual field examination often demonstrates enlargement of
the blind spot or peripheral or fascicular field defects, or,
more rarely, major defects that cause tunnel vision.
• No correlation has been established between tumor
size and tumor extension, or between the presence
of papilledema and the size of the visual field defect.

• Melanocytoma of the optic disk has been reported in
association with ocular melanocytosis,congenital
hypertrophy of the pigment epithelium, choroidal
nevus, and retinitis pigmentosa

Investigations
• Ophthalmoscopy generally sufficient to establish the
diagnosis of melanocytoma. The various
complementary investigations are performed
primarily to document the shape and extension of
the tumor and to detect possible complications.

• FLUORESCEIN ANGIOGRAPHY
• The part of the tumor that is invading the retina
remains hypofluorescent on all phases of fluorescein
angiography. A fine network of telangiectatic
capillaries is occasionally observed on the surface of
the tumor, and the filamentous edges are clearly
visible. In contrast, the part of the tumor invading
the choroid and situated underneath the pigment
epithelium has the same angiographic features as do
choroidal nevi.
• Fluorescein angiography can, however, detect or
confirm the presence of papilledema.

• B-SCAN OCULAR ULTRASOUND
• This examination can be used to measure
tumor thickness, but does not provide any
additional information.
• HEIDELBERG RETINA TOMOGRAPHY
• This examination can be used to precisely
measure the tumor surface in three
dimensions, and is therefore useful to detect
any changes in tumor volume

HRT, tumor apex/retinal
surface distance: 0.5 mm,
and study of the horizontal
and vertical profile of the
tumor

• Melanocytomas are composed mainly of intensely
pigmented, large polyhedral nevus cells. On
ultrastructural examination, the cytoplasm contains
few organelles, but a large number of giant
melanosomes. The nucleus is oval, generally without
a nucleolus, and there is a low nucleocytoplasmic
ratio

DIFFERENTIAL DIAGNOSIS
• Certain benign or malignant tumors of the choroid,
pigment epithelium, and optic disk, as well as certain
malformations, can mimic melanocytoma.
• The main differential diagnoses are choroidal
melanoma adjacent to and invading the optic disk,
primary melanoma of the optic disk, and adenoma or
adenocarcinoma of the juxtapapillary pigment
epithelium.

CLINICAL COURSE AND
COMPLICATIONS
• Melanocytomas of the optic disk are benign tumors
that generally remain stationary.
• Over a long period of observation, ∼40% of these
tumors show some slow growth,
• and ∼2% undergo malignant
transformation.
• Progression of a melanocytoma may be
complicated by partial or total visual loss
due to partial or total vascular occlusion
or nerve fiber necrosis, both of which
are secondary to pressure exerted by the
tumor.Wednesday, October 29, 2014 Department of Ophthalmology, JNMC 90

• Visual loss may occasionally be transient.
• In conclusion, although melanocytomas of the optic
disk are benign, they do present a low
risk of malignant transformation.
• They can also induce complications that
lead to severe loss of visual function,
and therefore require close surveillance.

Malignant astrocytomas

Malignant astrocytomas
• Rare neoplasms involving the anterior visual pathway
• almost always occur in adulthood, with a mean age
in the 60s
• they are minimally more common in males ( 1.3:1).
• Patients present with acute onset of pain and either
unilateral or bilateral visual loss, depending on
whether the optic nerve or the chiasm is initially
involved.

• With unilateral lesions, the second eye is invariably
involved within weeks.
• optic disc may be normal or pale at presentation ,
• but in cases of more anterior tumors, retinal venous
occlusive disease and disc edema are common
secondary to obstruction of the central retinal vein
within the intraorbital optic nerve

• An MRI scan most often shows diffuse intrinsic
enlargement and enhancement of the affected optic
nerves, chiasm, and optic tracts, with
inhomogenecity due to cystic spaces within the
tumor
• Occasionally, a large exophytic component may
encroach on the suprasellar cistern.
• Histologically, malignant optic nerve gliomas are
classified as anaplastic astrocytomas or glioblastoma
multiforme.

• Visual loss is severe and rapidly progressive.
• Treatment is rarely successful, although radiotherapy
and chemotherapy have been attempted, with
blindness usually developing 2-4 months after onset
of visual loss.
• The tumor is aggressively infiltrative, and death from
hypothalamic and brainstem involvement usually
occurs within 6- 12 months.

Astrocytic Hamartoma

• Astrocytic hamartomas of the retina, most common
in tuberous sclerosis and neurofibromatosis, may
take the form of so-called mulberry lesions.
• When located adjacent to the disc, they may closely
resemble optic disc drusens, and in early reports
they were termed giant drusen of the optic disc.
• In contrast to true optic disc drusens, disc
hamartomas originate at the disc margin, with
extension to the peripapillary retina arise in the inner
retinal layers and typically obscure retinal vessels
• may have a fleshy, pinkish component
• do not autofluoresce and may show tumorlike
vascularity on fluorescein angiography

Optic disc drusen
Astrocytic hamartoma

LYMPHORETICULAR TUMORS

LYMPHORETICULAR TUMORS
• Leukemia is a well-recognized cause of infiltrative
optic neuropathy.
• Vision loss may occur abruptly or over the course of
several days and is often bilateral.
• Many patients have a known history of leukemia at
the time of diagnosis.
• The fundus may show abundant retinal
hemorrhages and bilateral optic nerve edema

Fundus of the right eye of a 21-year-old man with acute lymphocytic
leukemia. There is severe edema of the nerve, exudates, and retinal
hemorrhages due to carcinomatous invasion of the optic nerve and its
sheath. (b) The left fundus shows much more severe retinal hemorrhages.

• Although a spinal tap is required to confirm the
diagnosis of leukemic invasion, an MRI can show
signs characteristic of infiltration.
Axial MRI through the orbits with gadolinium and fat suppression, revealing
enhancement and enlargement of the optic nerve sheaths bilaterally in a 21-year-old
man with acute lymphocytic leukemia. He had severe vision loss. (b) Coronal MRI
through the orbits showing a cross section of the enhancement and enlargement of
the optic nerve sheaths in the same patient.

METASTATIC TUMORS
OF THE OPTIC NERVE

METASTATIC TUMORS OF THE
OPTIC NERVE
• A number of carcinomas are known to metastasize to
the optic nerve.
• Patients frequently present with only mild vision loss
• neuro-imaging may initially appear normal or with
only subtle findings.
• Common primaries include breast, lung, and bowel
tumors.

• Renal carcinomas may also cause an optic
neuropathy.
• These tumors may infiltrate the optic nerve,
producing symptoms either as an initial
manifestation or as part of a more generalized
meningeal involvement.

Take home message
• Not infrequently, the ophthalmologist is calledon to determine
whether or not visualloss is caused by infiltrative or compressive
lesions of the anterior visualpathway. Thisis determined through
careful clinicalevaluation of the visualacuity, color vision, pupils,
visualfields, and fundus. Knowledge of the more common tumors will
assist the ophthalmologist in advising patients on a course of action,
and deciding which patients should be followed and which require
referral to neurosurgeons or radiation oncologists

Bibliography
# Anatomy and Physiology of the Eye byAK Khurana;2nd Edition
# Albert Jakobiec’s Principles and Practice of Ophthalmology. Section 14,
Section 17; 3rd Edition
# American Academy of Ophthalmology BCSC. Section 5; 2011-2012
# Yanoff & Duker Ophthalmology; 3rd Edition

THANKYOU
October 29, 2014 Department of Ophthalmology, JNMC 110


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