What do you see in the image?
This patient had renal cell cancer causing a paraneoplastic syndrome with deposition of red patches in the retinal pigment epithelium which in this case simulated blood. This deposition causes blindness and is thought to be stimulated by an immunoglobulin G fraction termed “cultured melanocyte elongation and proliferation factor.” Excision of the primary cancercan cause regression of the disease and plasmapheresis can stabilize visual loss.
This syndrome is called bilateral diffuse uveal melanocytic proliferation (BDUMP) . It can involve the eye, skin (where it appears as pigmented lesions) , and mucous membranes. It also can cause palmar fasciitis. It was first described in 1966 and has been reported in ovarian, uterine, lung, pancreas and breast cancers. It can precede the diagnosis of cancer by 3-12 months.
Teaching point: consider malignancy in individuals with vision loss.
Our patient had a long discussion with oncology and when he found out he could not be cured elected to forego chemo and simply do plasmapheresis to retain his vision.
Machemer R. On the pathogenesis of the flat malignant melanoma. Klin Monbl Augenheilkd. 1966; 148(5): 641-52. Zur Pathogenese des flachenhaften malignen Melanoms.
Miles SL, Niles RM, et al. A factor found in the IgG fraction of serum of patients with paraneoplastic bilateral diffuse uveal melanocytic proliferation causes proliferation of cultured human melanocytes. Retina 2012;32(9): 1959-66.
Sen J, Clewes AR, Quah SA, et al. Presymptomatic diagnosis of bronchogenic carcinoma associated with bilateral diffuse uveal melanocytic proliferation. Clin Experiment Ophthalmol. 2006;34(2):156-8.