Paraneoplastic vitelliform retinopathy: clinicopathologic correlation and review of the literature

Surv Ophthalmol. 2012 Nov;57(6):558-64. doi: 10.1016/j.survophthal.2012.02.004. Epub 2012 Jul 10.


Traditionally, the paraneoplastic retinopathies have been classified into two groups: melanoma-associated retinopathy (MAR) and cancer-associated retinopathy. MAR occurs in individuals with metastatic cutaneous or uveal melanoma and is characterized by nyctalopia, photopsias, and variable vision loss. In most cases, the fundus is essentially normal in appearance. More recently, there have been multiple reports of a MAR-like retinopathy with associated detachments of the retinal pigment epithelium and neurosensory retina. Such a clinical presentation has been termed paraneoplastic vitelliform retinopathy. We describe an 80-year-old man with metastatic cutaneous melanoma who developed paraneoplastic vitelliform retinopathy. For the first time, histopathology from enucleation specimens provides a clinicopathologic disease correlation with focal abnormalities in the inner nuclear and outer plexiform layers.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged, 80 and over
  • Autoantibodies / blood
  • Autoantigens / immunology
  • Blotting, Western
  • Carbonic Anhydrase II / immunology
  • Color Perception Tests
  • Electroretinography
  • Eye Enucleation
  • Fluorescein Angiography
  • Humans
  • Male
  • Melanoma / immunology
  • Melanoma / secondary*
  • Paraneoplastic Syndromes, Ocular / immunology
  • Paraneoplastic Syndromes, Ocular / pathology*
  • Retina / immunology
  • Retinal Neoplasms / diagnosis
  • Retinal Neoplasms / immunology
  • Retinal Neoplasms / secondary*
  • Skin Neoplasms / immunology
  • Skin Neoplasms / pathology*
  • Tomography, Optical Coherence
  • Visual Fields


  • Autoantibodies
  • Autoantigens
  • Carbonic Anhydrase II