OPTIC NERVE TUMOURS

PRESENTER- DR ADHEELA ABDULLA

MODERATOR- DR SHIKHA BASSI
DNB questions

 1.Neurofibromatosis-ocular manifestations.
Differential diagnosis of optic nerve glioma. (5+5) J2019

 2. What are the common tumours of optic nerve in adults?

Give clinical features to differentiate them clinically and give salient pathologic
features of these tumours. (3+2+5) J2013
OPTIC NERVE NEOPLASMS
PRIMARY METASTATIC
Optic nerve glioma –juvenile, benign Optic nerve metastasis

Malignant optic nerve glioma Lymphoma

Optic nerve sheath meningioma Leukemia

Melanocytoma Retinoblastoma

Vascular tumours Carcinomatous meningitis

CLINICAL FEATURES

COMPRESSIVE OPTIC NEUROPATHY

Monocular vision loss

Decreased colour vision Pain on eye movements
subacute, slowly progressive

Headache, facial numbness,

eyelid edema , orbital fullness decreased sensation of
smell
EXAMINATION

Decreased VA, Colour vision ,RAPD+ve

Associated ptosis, proptosis, eyelid retraction, lid lag,

ophthalmoplegia
OPTIC PATHWAY GLIOMA/
PRIMARY GLIOMA OF ANTERIOR
VISUAL PATHWAY
JUVENILE BENIGN PILOCYTIC
ASTROCYTOMA
 Most common primary tumour of optic nerve.
 1% of intracranial tumours.
 Involve optic nerve, chiasm or both
 70% -1st decade of life , 90%- 2nd decade- Any age.
Association with NF-1
Patients with NF-1 , prevalence of optic pathway glioma -7.8-21%

Patients with optic pathway glioma –prevalence of NF1-10-70%.

 Clinical course- highly variable- very slow growing

 non NF cases- worse prognosis.
 CLINICAL FEATURES
 Most common( 94%)- proptosis

 Vision loss (87.5%)- slowly progressive, painless.

 Young children- amblyopia

 Eye faces down with monocular elevation deficit

 Strabismus
 RAPD +ve
 Fundus-ONH edema /atrophy, optocilary shunt vessels
INTRACRANIAL EXTENSION

chiasm-bitemporal visual field defect, see-saw nystagmus.

Hypothalamus-precocious puberty.

Large tumors- obstructive hydrocephalus with raised intracranial

pressure
INVESTIGATION

 HVF- central scotoma, bitemporal VF defect can

occur

 CT/MRI- fusiform enlargement , kinking,

enhancement of optic nerve.

 Biopsy not required. HP pilocytic atrocytoma.

Ontrast enhanced axial orbital Ctscan-

right optic nerve enlarged&kinked
TREATMENT
Vision good,
stable radiographically NF1 Severe vision loss
cases- progression- chemotherapy- RT controversial
observation- spontaneous vincristine, carboplatin.
regression

Surgical excision- severe vision loss,

disfiguring proptosis- prevent Hydrocephalus- CSF shunting.
advancement to chiasm.
Case 1-optic nerve glioma

 35y male –left eye prominence & complete loss of

vision- 4-5months.

 BCVA- NO PL,Inferior dystopia with axial

proptosis,Hertels- 15/20,CT- LXT,EOM- full ,Pupil-
RRRL, RAPD+

 (Colour vision- deferred), right WNL

CsFundus-
OD- WNL, OS-temporal pallor, resolving disc edema1
 MRI BRAIN & ORBIT

Referred to oncologist- EBRT

Fusiform enlargement of left optic nerve extending up to optic canal-S/O optic

nerve glioma
Case 2- optic nerve glioma

 3y male child –OD prominence, deviation of eyeball outwards-

3months rapidly progressive, associated pain
 VA- not following light,RXT,EOM elevation deficit -3
 Hertels- 15/11,Pupil sluggishly reacting,RAPD+,optic disc pallor
 OS WNL
MRI brain & orbit

Fusiform enlargement of right optic nerve

up to optic canal indenting the globe
Chemotherapy –vincristine, carboplatin.
MALIGNANT OPTIC NERVE GLIOMA OF
ADULTHOOD
 Almost always occur in adulthood-60 years
 Rare tumour.
 High grade glial neoplasm.
 Agressively infiltarative- HT, brain stem- death
CLINICAL FEATURES
 Rapid vision loss.

 Acute onset peri orbital pain, pain on eye

movements, headache.

 Involve contralateral eye within weeks.

 Proptosis, conjunctival chemosis, NVG.

 Fundus- ONH normal/pale/edema. Retinal

obstruction –venous stasis retinopathy.

Optic disc edema

INVESTIGATION

 MRI- diffuse intrinsic enlargement and

enhancement of optic nerve , chiasm,optic tract.

 HP- anaplastic astrocytoma, glioblastoma

multiforme.

T1 W MRI-axial viewwith gadolinium-

thickening&enhancement of ON
OPTIC NERVE SHEATH
MENINGIOMA
 Arises from proliferation of meningioepithelial cells lining the sheath of infraorbital /intra
canalicular optic nerve.

 True primary ONSM less common, most meningioma are extension from intra cranial site.

 Impair axonal transport, interfere with pial blood supply to optic nerve.

 95% unilateral, bilateral & multifocal cases can ocuur with NF2.

 Middle aged females.Indolent growth.

CLINICAL FEATURES
 Diagnostic triad-
Painless slowly progressive monocular vision loss.

Optic atrophy.

Optocilary shunt/retinochoroidal collaterals.

• Double vision, transient visual

obscuration.
• Mild-proptosis, mild EOM defects.
• RAPD
INVESTIGATIONS

 CT scan- tram track sign, diffuse

enlargement of intra orbital optic nerve with
enhancement of optic nerve sheath.
 MRI- ring sign –coronal view- rightoptic
nerve sheath enhancement surrounding
normal optic nerve.

 Pattern of fusiform enlargement but no

kinking.
TREATMENT

 Fractionated RT – treatment of choice- salvageable vision.

 If no change in visual function, tumour size- observation.

 Surgery- not done, rarely if extends intracranially – contralateral vision loss

Case of optic nerve sheath meningioma

 38y male both eyes diminision of vision -4years,First right eye – 2y later left eye – gradual

 Associated mild headache, browache

 OD-BCVA-HM,EOM full, free, Pupil RRRL, RAPD+(Colour vision-deferred)

 OS BCVA- 2/60,EOM- full, Pupil RRRL, Colour vision-0/21

Fundus

ou- pale disc, tortuous vessels

HVF-

OD not responding, OS constricted

MRI brain orbit-

thickening and enhancing bilateral optic nerve sheath complex- s/o ONSM. Small olfactory groove
meningioma ,right cavernous sinus meningioma
TREAMENT

 Neurosurgery review – endoscopic decompression of optic nerve

and biopsy of right lesion followed by RT.
MELANOCTOMA OF OPTIC DISC
Brown or black lesion over optic disc with peripapillary extension to retina & choroid.

 Benign pigmented tumours composed of nevus cells.

 More common in dark skinned individuals.

 Asymptomatic and incidental.

 Neuroretinitis, Necrosis- throw seeds in vitreous., Rarely as optic neuritis

 Visual impairment -compression of optic nerve, CRVO, CRAO, tumour necrosis,
juxtapaillary choroid neovascularization, retinal exudation.

 HVF- enlargement of blind spot . Also arcuate scotoma, paracentral scotoma, quadrant
defect, altitudinal defect.

 Rarely malignant transformation.

 Case-melanocytoma &pituitary adenoma

 42year old female

 OD progressive diminision of vision x
6weeks
 VA-6/60,Pupil –sluggish,reduced Colour
vision
 OS WNL

Ref: Attiku, Y., Rishi, P. and Bassi, S., 2019. Coexisting Optic Disc
Melanocytoma and Pituitary Adenoma. Ocular Oncology and
Pathology, 5(5), pp.319-322. Fundus- OD elevated brown lesion over optic nerve head
2.2x1.8mm OS-mild temporal pallor
USG B scan

tumor with moderate surface reflectivity and homogenous internal reflectivity

Attiku, Y., Rishi, P. and Bassi, S., 2019. Coexisting Optic Disc Melanocytoma and Pituitary Adenoma. Ocular Oncology and Pathology, 5(5),
pp.319-322.
HVF

Attiku, Y., Rishi, P. and Bassi, S., 2019.

Coexisting Optic Disc Melanocytoma
and Pituitary Adenoma. Ocular Oncology
and Pathology, 5(5), pp.319-322.
  MRI- well circumscribed sellar  Pseudorosette &papillary pattern
mass

Attiku, Y., Rishi, P. and Bassi, S., 2019. Coexisting Optic Disc Melanocytoma and Pituitary Adenoma. Ocular Oncology and Pathology, 5(5), pp.319-
322.
PIGMENTED LESIONS OF OPTIC NERVE HEAD

 Choroidal melanoma with secondary invasion of optic nerve.

 Combined hamartoma of retina and RPE- pigmented ,little elevated, gliosis, vascular
tortuosity - OCT-ERM, retinal thickening
Vascular tumours involving optic nerve
head
cavernous hemangioma
Peripapillary hemangioblastoma
Capillary angioma
Well defined lobulated purplish
can compress optic nerve
Arises in intraconal space of orbit
Associated with VHL
Arise out of muscle cone
Compress optic nerve
Fleshy appearing lesions of ONH.
denovo or in patients with VHL disease.
Can cause vision loss in association with fluid
exudation or bleeding.
Cavernous hemangioma

T1W gadolinium enhanced MRI axial view-

lesion compress optic nerve
Capillary angioma

fleshy appearing lesion

METASTATIC OPTIC NERVE
NEOPLASMS
OPTIC NERVE METASTASIS

 Choroid, orbit –most common site of metastasis to eye.

 Breast, lung,stomach,prostate,pancreas.

 Fundus-visible mass invading ONH, vascular compression-CRVO,CRAO

 Neuroimaging- fusiform enlargement of optic nerve.

 High mortality
Case-bilateral optic disc metastasis from carcinoma
breast

 46year female

 Bilateral diminision of vision -3months

 H/O infiltrative duct adenocarcinoma breast- s/p right mastectomy, 6 cycles of chemotherapy- 5y
before

 After 4y- LN, bone metastasis-RT, chemotherapy.

Rishi, P., Dixit, A., & Verma, A. (2015). Bilateral optic disk metastasis from breast carcinoma. Indian Journal Of Ophthalmology, 63(5), 451. doi:
10.4103/0301-4738.159886
 OU NO PL,OU sluggish pupillary reaction.
 Infiltrative mass over ONH with intrinsic calcification, SRF

Rishi, P., Dixit, A., & Verma, A. (2015). Bilateral optic disk metastasis from breast carcinoma. Indian Journal Of Ophthalmology, 63(5), 451. doi:
10.4103/0301-4738.159886
 OCT-high reflectivity and posterior shadowing

Ref: Rishi, P., Dixit, A., & Verma, A. (2015). Bilateral optic disk metastasis
from breast carcinoma. Indian Journal Of Ophthalmology, 63(5), 451. doi:
10.4103/0301-4738.159886
VEP-extinguished response
LYMPHOMA

 Optic neuropathy rare

 Direct infiltration , part of lymphomatous meningitis.

 Vision loss by direct invasion via pial septae.

 Mass lesion in orbit.

Case of lymphoma involving optic nerve

 68y male-OD proptosis-gradual,progressive,

 VA-OU 6/9,N6, Hertles 19/13
 EOM OD abduction -1,elevation-1,adduction-1,depression full
 Insinuation possible all quadrants.
 OS WNL
 HVF-WNL
 MRI brain&orbit-intraconal mass enveloping optic nerve-restricted diffusion?
Lymhoproliferative.
 Incisional biopsy-extranodal NHL
 RT
 HVF-WNL
 MRI brain&orbit-intraconal mass enveloping optic nerve-restricted diffusion?
Lymhoproliferative.
 Incisional biopsy-extranodal NHL
 RT
5m follow-up -MRI

ill defined residual retrobulbar lesion partly encasing optic nerve-isointense in T1

LEUKEMIA
 Direct infiltration of ONH via pial septae – massive swelling.

 Vision loss- vascular compromise, chronic axoplasmic stasis

from swelling.

 RT – progressive vision loss & optic nerve involvement from

leukemia, lymphoma.

Peripapillary haemorrhage, venous distension and

tortuosity , retinal infiltration.
Case of retinoblastoma involving optic
nerve
 1y male child OD- white
reflex.

 OD- NO PL,buphthalmic
eye, OS-WNL

Large enhancing right intraocular mass lesion with optic nerve invasion
upto the canal(T1-intermediate signal,T2-mixed hypo&isointense signal,
restricted diffusion
USG B Scan
large retinochoroidal mass lesion entire vitreous cavity

CSF analysis- negative for tumour cells

chemotherapy(high dose vincristine,etoposide, carboplatin)
CARCINOMATOUS MENINGITIS
 Common cause of multiple cranial neuropathies secondary to multifocal seeding of leptomeninges.

 Most commonly- 3rd &6th CN, followed by 7th, 5th ,8th CN.

 Primary-Breast , lung.

 Mechanism of optic neuropathy-infiltrative, ischaemic,demyelinative, compressive.

 CSF-increased protein &neoplastic cells.

 MRI-thickening of optic nerve, abnormal meningeal enhancement.

references

 AAO neuro ophthalmology

 Liu volpe and Galetta neuro ophthalmology
 Attiku, Y., Rishi, P. and Bassi, S., 2019. Coexisting Optic Disc Melanocytoma and
Pituitary Adenoma. Ocular Oncology and Pathology, 5(5), pp.319-322.

 Rishi, P., Dixit, A., & Verma, A. (2015). Bilateral optic disk metastasis from breast
carcinoma. Indian Journal Of Ophthalmology, 63(5), 451. doi: 10.4103/0301-
4738.159886
Acknowledgement

 DR SRB mam
 DR Veena mam
 DR MVD
 DR KPI
THANK YOU