Long Survival and Preservation of Good Visual Acuity in a Patient with Bilateral Diffuse Uveal Melanocytic Proliferation

Ocul Oncol Pathol. 2019 Jan;5(1):75-78. doi: 10.1159/000488454. Epub 2018 Jun 19.

Abstract

Purpose: To report a patient with bilateral diffuse uveal melanocytic proliferation (BDUMP) due to a lung carcinoma, who survived 9.5 years with preservation of good visual acuity after cataract surgery and curative chemotherapy with neoadjuvant radiation therapy.

Methods: Clinical review of a patient with BDUMP.

Results: We report the case of a 64-year-old man presenting with bilateral visual deterioration. Ophthalmological examination showed bilateral cataract, a small elevated non-pigmented tumor in the iris and multiple elevated, diffuse round pigmented choroidal lesions. Based on the clinical presentation, the diagnosis of BDUMP was suspected. After referral to an internal medicine specialist, a poorly differentiated non-small cell lung carcinoma was diagnosed and treated with chemotherapy followed by irradiation. After 3 years, spontaneous depigmentation of the choroidal lesions occurred. According to the literature, the mean survival of patients with BDUMP is 11.6 months. Our patient, however, survived for 9.5 years with preservation of good visual acuity after cataract extraction.

Conclusion: This case illustrates that prolonged survival and preservation of useful visual acuity is possible in BDUMP. Cataract surgery should be considered in selected patients, and depigmentation of the lesions may occur during long-term follow-up.

Keywords: Bilateral diffuse uveal melanocytic proliferation; Iris; Paraneoplastic syndrome; Uveal tumor.