A Case of Bilateral Diffuse Uveal Melanocytic Proliferation in the Setting of Urothelial Carcinoma of the Ureter: A Failed Response to Plasmapheresis

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome. Diagnosis can be difficult and treatment guidelines are ill-defined. A 73-year old man with poorly differentiated high grade urothelial carcinoma of the ureter metastatic to the liver, lungs, lymph nodes, and pelvis treated with chemotherapy and immunotherapy reported gradual vision loss. Optical coherence tomography (OCT) demonstrated intraretinal and subretinal fluid with hyperreflective subretinal deposits and focal, deeply pigmented choroidal lesions. Irregular hyperpigmentation of the retinal pigment epithelium (RPE) with a leopard spot pattern on fundus autofluorescence (FAF) supported the diagnosis of BDUMP. Multiple plasmapheresis sessions did not result in sustained clinical improvement. [Ophthalmic Surg Lasers Imaging Retina 2022;53(6): 350-353.]

Automated summary

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome that presents with gradual vision loss. This case report describes a 73-year-old man with metastatic urothelial carcinoma who developed visual symptoms. Ophthalmic examination revealed fluid accumulation, subretinal deposits, and irregular hyperpigmentation, consistent with the diagnosis of BDUMP. Despite undergoing multiple plasmapheresis sessions, the patient did not experience sustained clinical improvement.