First Author: C.Menezes PORTUGAL
Co Author(s): J. Martins R. Carvalho C. Teixeira M. H Abreu A. Rodrigues M. Soares 0 0 0 0 0 0 0 0 0
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To report a markedly asymmetric presentation BDUMP and its clinical and imaging response to the palliate chemotherapy of the primary tumor.
1- Department of Ophthalmology - Hospital Pedro Hispano, Matosinhos, Portugal 2- Department of Oncology – Insituto Português de Oncologia, Porto, Portugal
Clinical case report.
BDUMP is a rare paraneoplastic syndrome associated with systemic carcinomas. It is characterized by the proliferation of spindle shaped melanocytes within the choroid and has a bad visual prognosis and low life expectancy. A 67-year-old man was referred to our department due to OD loss of vision and cataract. He was a heavy smoker and reported constitutional symptoms in the last 6 months. On examination, BCVA was 0.2 in OD and 1.0 in OS. Biomicroscopy revealed a mild corticonuclear cataract in OD. Ophthalmoscopy showed, in OD, a slightly elevated macular lesion reminding a super-nevus, areas of segregating RPE in a reticular pattern and a coexistent exsudative macular retinal detachment; and, in OS, a round hypopigmented area in the papillomacular bundle. The fluorescein angiography, autofluorescence and OCT corroborated the diagnosis of BDUMP: advanced in OD and initial in OS. A pulmonary adenocarcinoma (cT4NOM1) was diagnosed and the patient underwent palliate chemotherapy. The systemic response was good with functional status improvement. BCVA in OD increased to 0.3, the pigmentation reduced and the exsudative detachment resolved. OS remained stable. 5 months later, 13 months after the diagnosis, the patient died and, according to his family, experienced bilateral loss of vison before his death.
The clinical presentation of BDUMP can be markedly asymmetric. The partial regression and stabilization of the BDUMP lesions enhances the paraneoplastic nature of this disease. In the absence of a curative treatment, the natural history of BDUMP encloses a poor visual prognosis and low life expectancy.