Bilateral diffuse uveal melanocytic proliferation and uterine cancer. A case report

Abstract

ABSTRACT.Purpose: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP), a rare paraneoplastic syndrome causing visual loss in patients with systemic carcinoma. Results: A 70-year-old woman developed visual symptoms 13 months after surgery and local irradiation therapy for uterine cancer. Following bilateral external beam irradiation supplemented with subsequent drainage of subretinal fluid in the left eye, the visual acuity improved from 0.01 to 0.15 in this eye only. The visual acuity remained at this level until she died 4 1/4 years after the onset of eye symptoms. Conclusion: This is the fourth case that survived longer than 24 months after the onset of visual symptoms of the 22 previously reported cases with BDUMP. It demonstrates that radiotherapy may have a vision-preserving effect in this group of patients. The patient also developed two different paraneoplastic phenomena -a nephrotic syndrome before and BDUMP after treatment for uterine cancer.Key words: bilateral diffuse uveal melanocytic proliferation -drainage of subretinal fluid -irradiation therapy -paraneoplastic syndrome -uterine cancer -uvea. Acta Ophthalmol. Scand. Acta Ophthalmol. Scand. 2000: 78: 366-368 Copyright c Acta Ophthalmol Scand 1999. ISSN 1395-3907 B ilateral diffuse uveal melanocytic proliferation is a rare paraneoplastic ocular syndrome that causes visual loss in patients with systemic carcinoma. The main characteristics are: Multiple round, red patches at the level of the retinal pigment epithelium, a striking pattern of multifocal areas of early hyperfluorescence corresponding with these patches, proliferation of choroidal nevus-like lesions as well as diffuse thickening of the uveal tract, exudative retinal detachment, and rapidly developing cataract (Barr et al. 1982;Gass et al. 1990). In addition glaucoma, dilated episcleral vessels, iridocyclitis, shallow anterior chambers, ciliary body cysts, and iridodonesis have been reported (Murphy et al. 1997). The syndrome frequently causes visual symptoms prior to the presentation of the primary tumour (Gass et al. 1990) and is usually lethal within 12 to 24 months (Gass 1987). Case ReportA 70-year-old woman was treated for a uterine cancer stadium 1B with oophorohysterectomy and additional local irradiation. Before surgery the patient developed a nephrotic syndrome caused by a biopsy-proven membranoproliferative glomerulonephritis. After treatment the renal symptoms disappeared, and the nephrotic syndrome was interpreted as a paraneoplastic phenomenon.One year later she was referred for an eye examination at the local hospital due to reduced vision, and the visual acuity turned out to be 0.5 in both eyes. Dilated episcleral vessels and a bilateral glaucoma with shallow anterior chambers were found. The glaucoma in the left eye was refractory to local antiglaucomatous treatment and systemic acetazolamid. YAG-iridotomy performed subsequently in the left eye revealed a substantial IOP-reduction. During the following months a rapid cataract development occurre...

The potential role of plasma exchange as a treatment for bilateral diffuse uveal melanocytic proliferation: A report of two cases

“…In addition, in both patients, the sustained treatment effect may be due in part to continued chemotherapy treatment of the underlying malignancy. Finally, in Patient 2, the use of corticosteroids may also play a role in the sustained response to PE, a finding that has been reported in some [16,23] but not all [7,11,17,19] patients treated with corticosteroids.…”

“…Other patients have been treated with bilateral enucleation. Corticosteroids and other immunosuppressants have been suggested as possible treatment modalities [21] but have been reported in only a few cases [7,11,16,17,19,23]. Variable responses have been reported in these cases with four patients demonstrating no benefit [7,11,17,19] and two demonstrating responses [16,23].…”

“…The disorder presents with rapid-onset vision loss in patients in their 50s through 80s. The ocular findings and vision loss often appear before recognition of the underlying malignancy [1][2][3][4][7][8][9][10]12,15,16,21,22], but may become apparent after diagnosis of the malignancy in up to half of cases [3][4][5][6][8][9][10]13,[17][18][19][23][24][25]. BDUMP is most commonly associated with a gynecologic carcinoma in women [8][9][10]12,[17][18][19]21,23,24] and lung [2,3,4,15,16,25] or pancreatic [1,3,10,22] cancer in men.…”

Multimodal imaging of bilateral diffuse uveal melanocytic proliferation associated with an iris mass lesion

“…Various treatments for BDUMP, including ocular radiation [1, 3, 19, 32], subretinal fluid drainage [30] and corticosteroids [15, 16, 24, 35] have been tried without success. Recently, plasmapheresis has been shown to improve visual acuity in few case reports.…”

Bilateral diffuse uveal melanocytic proliferation associated with the recurrence of a Bartholin gland carcinoma

“…Atrophy, pigmented and unpigmented elevated lesions and general inflammatory signs may be part of primary ocular melanoma; choroidal metastasis; large cell lymphoma; leukaemic retinopathy; acute, posterior multifocal placoid pigment epitheliopathy and benign lymphoid hyperplasia, which are common specific entities. However, although BDUMP syndrome occurs bilateral, it resembles and may mimic variations of such entities, and differentiation can be difficult 7 8…”