PMID- 25174893 OWN - NLM STAT- MEDLINE DCOM- 20150204 LR - 20141203 IS - 1879-1891 (Electronic) IS - 0002-9394 (Linking) VI - 158 IP - 6 DP - 2014 Dec TI - Paraneoplastic cloudy vitelliform submaculopathy in primary vitreoretinal lymphoma. PG - 1253-1261.e2 LID - S0002-9394(14)00533-9 [pii] LID - 10.1016/j.ajo.2014.08.031 [doi] AB - PURPOSE: To describe the nature and evolution of paraneoplastic cloudy vitelliform submaculopathy in patients with primary vitreoretinal lymphoma and propose a mechanism for its development and course. DESIGN: Retrospective, observational case series. METHODS: Three patients presenting with unilateral cloudy vitelliform submaculopathy based on clinical examination, fundus autofluorescence, fluorescein angiography, and spectral-domain optical coherence tomography (SD OCT) imaging and ultimately diagnosed with primary vitreoretinal lymphoma and/or primary central nervous system lymphoma were analyzed. RESULTS: In all 3 patients, cloudy vitelliform submaculopathy appeared with hazy indistinct yellow subretinal material resembling the vitelliform lesions found in acute exudative paraneoplastic polymorphous vitelliform maculopathy, although with less distinct appearance and without intense hyper-autofluorescence. In all 3 patients, cloudy vitelliform submaculopathy was transient, showed spontaneous regression within 3 months, and preceded the diagnosis of lymphoma, suggestive of a paraneoplastic process. The diagnosis of primary vitreoretinal lymphoma and/or primary central nervous system lymphoma was made within 6 months with classic features of new intraretinal or sub-retinal pigment epithelium infiltration of lymphoma in the peripheral retina (n = 2) and hyperintense lesions on brain magnetic resonance imaging (n = 2). With SD OCT imaging, the cloudy vitelliform subretinal lesions appeared as hyperreflective debris above the retinal pigment epithelium band in all 3 eyes, and were associated with an irregularly thickened and rippled retinal pigment epithelium band in 2 eyes. Resolution of the cloudy submacular lesions resulted in outer retinal atrophic changes in all 3 eyes. CONCLUSION: Paraneoplastic cloudy vitelliform submaculopathy, a form of lymphoma-associated retinopathy, can precede the diagnosis of primary vitreoretinal lymphoma or primary central nervous system lymphoma and can regress spontaneously, leaving outer retinal abnormalities. CI - Copyright (c) 2014 Elsevier Inc. All rights reserved. FAU - Pang, Claudine E AU - Pang CE AD - Vitreous 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. Electronic address: claudine.e.pang@gmail.com. FAU - Shields, Carol L AU - Shields CL AD - Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. FAU - Jumper, J Michael AU - Jumper JM AD - West Coast Retina Medical Group, San Francisco, California. FAU - Yannuzzi, Lawrence A AU - Yannuzzi LA AD - Vitreous 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; Department of Ophthalmology, New York University, New York, New York. LA - eng PT - Case Reports PT - Journal Article PT - Observational Study PT - Research Support, Non-U.S. Gov't DEP - 20140828 PL - United States TA - Am J Ophthalmol JT - American journal of ophthalmology JID - 0370500 SB - IM MH - Aged MH - Female MH - Fluorescein Angiography MH - Humans MH - Lymphoma, Non-Hodgkin/*diagnosis/physiopathology MH - Male MH - Middle Aged MH - Optical Imaging MH - Paraneoplastic Syndromes/*diagnosis/physiopathology MH - Remission, Spontaneous MH - Retinal Neoplasms/*diagnosis/physiopathology MH - Retrospective Studies MH - Tomography, Optical Coherence MH - Visual Acuity/physiology MH - Vitelliform Macular Dystrophy/*diagnosis/physiopathology MH - Vitreous Body/*pathology EDAT- 2014/09/02 06:00 MHDA- 2015/02/05 06:00 CRDT- 2014/09/02 06:00 PHST- 2014/06/17 00:00 [received] PHST- 2014/08/24 00:00 [revised] PHST- 2014/08/25 00:00 [accepted] PHST- 2014/09/02 06:00 [entrez] PHST- 2014/09/02 06:00 [pubmed] PHST- 2015/02/05 06:00 [medline] AID - S0002-9394(14)00533-9 [pii] AID - 10.1016/j.ajo.2014.08.031 [doi] PST - ppublish SO - Am J Ophthalmol. 2014 Dec;158(6):1253-1261.e2. doi: 10.1016/j.ajo.2014.08.031. Epub 2014 Aug 28.