1. MASQUERADE
SYNDROMES :
NEOPLASMS Bipin Bista
Resident 2ND year
Ophthalmology
National medical College
& Teaching Hospital

2. Introduction
 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-
saving.

3. Primary Neoplasms
 Primary Intraocular (Vitreoretinal)
Lymphoma (PIOL/PVRL)
 Typically extranodal Non-Hodgkin’s
Lymphoma , diffuse large B cell lymphoma
with or without simultaneous CNS
involvement.
 Challenging malignancy
 6th – 7th decade.

4. PVRL – Clinical Features
 Blurred vision
 Floaters
 Vitreous cells
 Vitreous haze
 Anterior chamber
inflammation
 Keratic precipates
 Exudative RD or RPE
detachment
 PCNSL in HIV is 2-6 %, at least 1,000
timesmore thanaveragepopulation.

5. Diagnosis
 Definitive requires identification of malignant
lymphoma cells in ocular specimens.
 Specimens : Vitreous, aqueous , or chorioretinal
biopsy.
 Crucial to rule out with pathologist , MRI and LP
with CSF cytology.
 Chorioretinal biopsies & enucleation shows
lymphoma cells between RPE & Bruch’s
membrane
 Undiluted fresh vitreous – cell culture &
transported for processing.
 Cytology : atypical lymphoid cells with scanty
basophilic cytoplasm

7. Contd..
 High ratio of
Interleukin -10 to
Interleukin -6 (pro-
inflammatory
cytokine) is
suggestive of PVRL
& ratio is greater
than 1 has a
reasonable
sensitivity &
specificity but is not
diagnostic.

8. Treatment
 Multidisciplinary approach
 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
temozolomide.
 Use of Anti- CD 20 monoclonal Ab.

9. Primary Choroidal Lymphomas and
Lymphoid Hyperplasia
 Typically extranodal marginal zone, MALT
lymphomas.
 Rare
 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.

10. Melanoma
 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
retinal detachment.
 Treatment enucleation, brachytherapy or
charged particle radiation.

11. Secondary neoplasms and
metastasis
 Lymphoma & leukemia
 Usually confined to choroid.
 Less diagnostic challenge since most have
known history.
 Exhibit creamy choroidal/subretinal infiltrates.
 Most are MALT associated with good
prognosis (10 yr survival)
 Bad prognosis : Peripheral T cell lymphoma,
Mycosis fungoides.

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
disturbances
 ERG & VF show scotomas and decreased rod
and cone responses.
 CAR is associated with Oat cell Carcinomas

14. PNS
 MAR is a common complication of metastatic
cutaneous melanoma.
 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
MELANOCYTIC PROLIFERATION
 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
progressive cataracts.
 Histopathology shows melanocytic
hyperplasia.

16. CONCLUSION
 The most important step to diagnose
neoplastic masquerade syndrome is clinical
suspicion.
 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
multidisciplinary approach.

17. Reference:
- Nussenblatt and whitecup 4th edition
- Myron yanoff 4th edition
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