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A 5-year-old girl with Aicardi syndrome and microphthalmia with cyst of the OD presented with progressive enlargement of the cyst causing pain. Microophthalmia with inferior cyst (35 × 25 × 12 mm) was noted at birth, and Aicardi syndrome was diagnosed at 10 months by the presence of the classic triad of callosal agenesis, infantile spasms, and chorioretinal lacunae. She underwent enucleation with cyst resection, and subsequent reconstruction with a dermis fat graft. Histopathologic study revealed adenocarcinoma of the pigmented ciliary epithelium. Full-body metastatic workup was negative. Adenocarcinoma of the pigmented ciliary epithelium is an extremely rare eye tumor with only 4 documented cases in the literature, none arising in a microophthalmic eye with cyst. Aicardi syndrome is also a rare disease that has been associated with increased incidence of malignancy and ocular abnormalities, but has never been described in association with microophthalmia with cyst or with adenocarcinoma of the pigmented ciliary epithelium. Herein, the authors present a review of the case and relevant literature.

We report a case of adenocarcinoma of the pigmented ciliary epithelium arising in a phthisical eye. A 92-year-old man who initially presented with severe ocular pain had calcification extending from the posterior pole to ciliary body on B-scan ultrasonography to a degree not previously reported. We highlight the importance of screening for intraocular neoplasms in adults with a long-standing phthisical eye., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A case of a 64-year-old man with bilateral diffuse uveal melanocytic proliferation is presented. Bilateral diffuse uveal melanocytic proliferation is a rare paraneoplastic disorder where an underlying malignancy causes bilateral blindness by uveal thickening, serous retinal detachment, and rapid cataract formation. The ocular symptoms and signs herald the onset of this disease, which leads to death in most cases within about 1 year. Including the present case, our literature review reveals that a total of 28 cases of bilateral diffuse uveal melanocytic proliferation have now been reported. Several different malignancies have been associated with bilateral diffuse uveal melanocytic proliferation, but ovarian carcinoma in women and lung and suspected pancreatic carcinoma in men are the most common. Our case is the first to be proven at autopsy to be associated with pancreatic carcinoma. The underlying mechanism remains to be identified, as numerous endogenous factors may regulate the proliferation of uveal melanocytes. Consideration of this entity during clinical examination may lead to an earlier diagnosis of malignancy and an improved prognosis.