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Paraneoplastic cloudy vitelliform submaculopathy in primary vitreoretinal lymphoma
Claudine E Pang1, Carol L Shields2, J Michael Jumper3
1Vitreous Retina Macula Consultants of New York, New York, New York; LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York.
American Journal of Ophthalmology
|September 2, 2014
Summary
Paraneoplastic cloudy vitelliform submaculopathy, a rare sign of lymphoma-associated retinopathy, can appear before lymphoma diagnosis and resolve spontaneously. This condition may lead to lasting outer retinal damage.
Area of Science:
- Ophthalmology
- Oncology
- Retinal Diseases
Background:
- Primary vitreoretinal lymphoma (PVRL) and primary central nervous system lymphoma (PCNSL) are rare B-cell malignancies.
- Paraneoplastic retinopathy can manifest as an early sign of these lymphomas.
Observation:
- This study analyzed three patients with unilateral cloudy vitelliform submaculopathy.
- Clinical examination, fundus autofluorescence, fluorescein angiography, and SD OCT imaging were used.
- Patients were ultimately diagnosed with PVRL and/or PCNSL.
Findings:
- Cloudy vitelliform submaculopathy presented as indistinct yellow subretinal material, transient and self-resolving within 3 months.
- This submacular change preceded lymphoma diagnosis by up to 6 months.
- SD OCT revealed hyperreflective debris above the retinal pigment epithelium, with RPE thickening and ripples in some cases.
Implications:
- Cloudy vitelliform submaculopathy may serve as an early indicator of underlying lymphoma.
- Spontaneous regression of these lesions can occur, but often results in outer retinal atrophy.
- Early detection and diagnosis of lymphoma are crucial for patient management.
