title: Paraneoplastic cloudy vitelliform submaculopathy in primary vitreoretinal lymphoma.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: Claudine E
authors.initials: CE
authors.lastname: Pang
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.affiliation: West Coast Retina Medical Group, San Francisco, California.
authors.forename: J Michael
authors.initials: JM
authors.lastname: Jumper
authors.affiliation: 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.
authors.forename: Lawrence A
authors.initials: LA
authors.lastname: Yannuzzi
mesh_terms: D000368:Aged; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D061848:Optical Imaging; D010257:Paraneoplastic Syndromes / Q000175:diagnosis* / Q000503:physiopathology; D012075:Remission, Spontaneous; D019572:Retinal Neoplasms / Q000175:diagnosis* / Q000503:physiopathology; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology; D057826:Vitelliform Macular Dystrophy / Q000175:diagnosis* / Q000503:physiopathology; D014822:Vitreous Body / Q000473:pathology*