Bilateral diffuse uveal melanocytic proliferation associated with extraocular cancers - Review of a process particularly associated with gynecologic cancers

We reviewed cases of a paraneoplastic syndrome in which uveal melanocytes p roliferated and led to blindness. Eighteen cases were derived from the lite rature, 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 a ntedated those of the inciting tumor. Most of the inciting tumors were poor ly differentiated carcinomas. The most common tumors were from the female g enital tract (ovary and uterus) among the women patients and from the lung among the men. Tumors from the breast were rare tone possible case), and tu mors of the prostate were conspicuously absent. All five inciting tumors wh ose 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 gene ral pathologists are not aware of this unique paraneoplastic syndrome. Our report is the first to document a statistically significant association bet ween this syndrome and gynecologic cancers.