Bilateral diffuse uveal melanocytic proliferation associated with extraocular cancers: review of a process particularly associated with gynecologic cancers

Am J Surg Pathol. 2001 Feb;25(2):212-8. doi: 10.1097/00000478-200102000-00009.


We reviewed cases of a paraneoplastic syndrome in which uveal melanocytes proliferated and led to blindness. Eighteen cases were derived from the literature, and two were taken from our institution. The average patient age at the time of the diagnosis was 63 years (range, 34-89 years). There were 13 women and 7 men. In approximately half of the cases, the ocular symptoms antedated those of the inciting tumor. Most of the inciting tumors were poorly differentiated carcinomas. The most common tumors were from the female genital tract (ovary and uterus) among the women patients and from the lung among the men. Tumors from the breast were rare (one possible case), and tumors of the prostate were conspicuously absent. All five inciting tumors whose histopathology was reviewed expressed neuron-specific enolase, but none prominently expressed antigens more specific for neuroendocrine carcinomas such as chromogranin or synaptophysin. It is our experience that many general pathologists are not aware of this unique paraneoplastic syndrome. Our report is the first to document a statistically significant association between this syndrome and gynecologic cancers.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adenocarcinoma / complications
  • Adenocarcinoma / secondary*
  • Cell Division
  • Endometrial Neoplasms / complications
  • Endometrial Neoplasms / pathology*
  • Fatal Outcome
  • Female
  • Humans
  • Hyperplasia
  • Melanocytes / pathology*
  • Middle Aged
  • Paraneoplastic Syndromes / complications
  • Paraneoplastic Syndromes / pathology*
  • Uveal Diseases / etiology
  • Uveal Diseases / pathology*