Definition : Simulation of an inflammatory
condition by a neoplastic process.
Rare group of disorder
First used in the Ophthalmology by Theodore
in 1967 to describe conjunctival carcinoma.
Infectious masquerade are potentially curable
while neoplastic masquerade can be life-
3. Primary Neoplasms
Primary Intraocular (Vitreoretinal)
Typically extranodal Non-Hodgkin’s
Lymphoma , diffuse large B cell lymphoma
with or without simultaneous CNS
6th – 7th decade.
4. PVRL – Clinical Features
Exudative RD or RPE
PCNSL in HIV is 2-6 %, at least 1,000
Definitive requires identification of malignant
lymphoma cells in ocular specimens.
Specimens : Vitreous, aqueous , or chorioretinal
Crucial to rule out with pathologist , MRI and LP
with CSF cytology.
Chorioretinal biopsies & enucleation shows
lymphoma cells between RPE & Bruch’s
Undiluted fresh vitreous – cell culture &
transported for processing.
Cytology : atypical lymphoid cells with scanty
High ratio of
Interleukin -10 to
Interleukin -6 (pro-
suggestive of PVRL
& ratio is greater
than 1 has a
specificity but is not
Sensitive to radiotherapy and chemotherapy
but the outcomes are still poor.
Mainstay of therapy : Methotrexate with or
without RT has been mainstay of therapy with
an overall survival of 24-40 months.
Cytarabine, Arabinoside, glucocorticoids and
Use of Anti- CD 20 monoclonal Ab.
9. Primary Choroidal Lymphomas and
Typically extranodal marginal zone, MALT
Not associated with CNS
Multifocal cream choroidal infiltrates
Choroidal thickening on USG
Extraocular extension leading to orbital mass
or salmon colored conjunctival mass
Responds to corticosteroids & low dose RT.
Most frequent neoplasm of the eye.
Seen in early 60’s mainly in white population.
Presents with iridocyclitis, dilated episcleral
vessels (sentinel vessels)
Choroidal granuloma, sectoral cataract,
posterior scleritis, secondary glaucoma or
Treatment enucleation, brachytherapy or
charged particle radiation.
11. Secondary neoplasms and
Lymphoma & leukemia
Usually confined to choroid.
Less diagnostic challenge since most have
Exhibit creamy choroidal/subretinal infiltrates.
Most are MALT associated with good
prognosis (10 yr survival)
Bad prognosis : Peripheral T cell lymphoma,
12. Metastatic Carcinoma
Particular to the choroid
Lung and breast carcinoma
b/l choroidal involvement with multifocal grey-
yellow lesions which are flat , can be
associated with vitritis, Exudative RD’s ‘
leopard spot’ pattern and papilloedema
CNS metastasis have poor prognosis.
13. Paraneoplastic syndromes
Presents with photopsia, dyschromatopsia,
vision loss, nyctalopia, photoaversion, mild
vitritis and less frequently macular edema, but
fundus can look normal in early stage.
Vascular attenuation, optic nerve pallor, RPE
ERG & VF show scotomas and decreased rod
and cone responses.
CAR is associated with Oat cell Carcinomas
MAR is a common complication of metastatic
ERG shows negative b-wave with realtively
preserved photopic responses.
Retinopathy precedes cancer in CAR while it
follows the diagnosis of melanoma by months
to years in MAR
15. BILATERAL DIFFUSE UVEAL
Occurs in patients with occult carcinoma
Characterized by multiple subtle sub retinal
reddish-brown lesions that show hyper
fluorescence on angiogram, exudative RD,
thickening of the uvea, mild uveitis and rapidly
Histopathology shows melanocytic
The most important step to diagnose
neoplastic masquerade syndrome is clinical
A thorough history, systemic review and
careful ophthalmic exam aided by appropriate
ancillary testing are required
Early recognition of the malignancy is crucial
for both vision saving and life saving
outcomes, which can be achieved through