Bilateral diffuse uveal melanocytic proliferation: Case report and literature review

Abstract

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic intraocular disease that causes progressive visual loss in patients driven by an IgG factor associated with an underlying malignancy. Characteristic ocular findings include exudative retinal detachment, rapid cataract formation and uveal melanocytic tumours. The awareness and documentation of BDUMP has increased during the past decade, and the increasing amount of data collected demonstrates the effect of treatment with plasmapheresis and the value of diagnostic tools in BDUMP such as genetic and immunologic investigations. The literature of BDUMP has not been reviewed since 2003, and there is a growing need for an updated review on diagnosis and management of BDUMP. We review the literature and report a case of BDUMP with a white ciliary body tumour, iris rubeosis, increased iris pigmentation and cataract.

Diagnosis of bilateral diffuse uveal melanocytic proliferation unveils primary gastric adenocarcinoma: a case report

“…Treatment of BDUMP primarily targets the primary malignancies, including local resection, radiation and systemic chemotherapy. Since systemic factors elicited by primary malignancies is considered involved in the pathogenesis of BDUMP, this could possibly explain the improvement of visual symptoms in some cases after these treatments targeting the malignancy [1]. Plasmapheresis can theoretically remove plasma CMEP, but with variable effectiveness [6].…”

“…Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome (PNS) affecting the eye, with around 60 cases reported [1]. There are five cardinal signs: (1) multiple, round or oval, subtle, patches at the level of the retinal pigmented epithelium (RPE) in the posterior fundus; (2) multifocal areas of early hyper-fluorescence corresponding with these patches; (3) multiple, slightly elevated, pigmented and non-pigmented uveal melanocytic tumors, as well as evidence of diffuse thickening of the uveal tract; (4) exudative retinal detachment; and (5) rapid progression of cataract [2].…”

“…There are five cardinal signs: (1) multiple, round or oval, subtle, patches at the level of the retinal pigmented epithelium (RPE) in the posterior fundus; (2) multifocal areas of early hyper-fluorescence corresponding with these patches; (3) multiple, slightly elevated, pigmented and non-pigmented uveal melanocytic tumors, as well as evidence of diffuse thickening of the uveal tract; (4) exudative retinal detachment; and (5) rapid progression of cataract [2]. It is thought either a substance secreted by the tumor or an antibody stimulated by the tumor, that causes benign proliferation of choroidal melanocytes [1]. Female urogenital (69%) and male lung carcinomas (52%) were reported more often, with sporadic cases including pancreatic, esophageal, breast, hepatocellular, Bartholin gland and renal cell carcinoma and central nervous system lymphoma [1].…”

“…It is thought either a substance secreted by the tumor or an antibody stimulated by the tumor, that causes benign proliferation of choroidal melanocytes [1]. Female urogenital (69%) and male lung carcinomas (52%) were reported more often, with sporadic cases including pancreatic, esophageal, breast, hepatocellular, Bartholin gland and renal cell carcinoma and central nervous system lymphoma [1]. Herein, we reported this case of BDUMP patient whose primary malignancy was gastric adenocarcinoma verified with gastroscopy and subsequent pathological test.…”

Bilateral diffuse uveal melanocytic proliferation: A case report and literature review

“…A review published in 2017 reported that the incidence of BDUMP was signi cantly increased during the past forty years [7]. After that review published, more than 10 cases of BDUMP were reported in the last three years [4,6,[8][9][10][11][12][13][14][15], and it was seemed that the incidence of this disease was much higher than before.…”