Vision Loss from Choroidal and Pituitary Metastases Secondary to Renal Cell Carcinoma: A Case Report.

A 62-year-old male with a history of metastatic clear cell renal cell carcinoma (ccRCC) presented with decreased vision to 20/50 in the left eye. Fundus examination revealed an elevated, amelanotic mass lesion in the superotemporal macula, without involvement of the central macula by subretinal fluid or tumour. Given incongruity between the fundus findings and the degree of visual impairment, visual field testing was obtained, revealing a bitemporal hemianopia. Magnetic resonance imaging demonstrated optic chiasm compression by a pituitary mass, which had previously been overlooked on computed tomography imaging. Biopsy confirmed metastatic ccRCC to the pituitary, which presented simultaneously with the presumed choroidal metastasis.

Recurrent melanoma arising from sclera.

Purpose: To report a case of recurrent malignant melanoma suspected to have arisen from intrascleral melanocytic cells. Observations: En bloc removal of melanoma was performed with iridocyclectomy in a 46-year-old Caucasian male. Histopathologic examination confirmed a diagnosis of malignant melanoma in the subconjunctival space, which was presumed to have arisen from the sclera and extended both intraocularly and subconjunctivally. 15 years later, a pigmented limbal lesion near the site of the previous iridocyclectomy was excised by lamellar sclerectomy. Histopathology showed a proliferation of pigment-containing cells with atypical nuclei consistent with recurrent melanoma. Conclusions and Importance: We report a case of recurrent melanoma that we suspect arose from intrascleral melanocytes, extended both intraocularly and subconjunctivally, and recurred 15 years following initial excision.

Treatment of Refractory Cystoid Macular Edema with Pegylated Interferon Alfa-2A: A Retrospective Chart Review.

Purpose: To determine the efficacy of pegylated interferon alfa-2A in the treatment of refractory inflammatory cystoid macular edema (CME)Methods: Retrospective chart reviewResults: Treatment with pegylated interferon alfa-2A led to an improvement in CME in all eyes of seven included patients, with a mean decrease in CMT from 478 µm to 310 µm (p < .05). The vision in one patient did not improve due to preexisting retinal atrophy. All other eyes showed improvement in vision, with a mean improvement in best LogMAR visual acuity from +0.59 to +0.28 (p < .05). The treatment effect was sustained with low-dose treatment every 2 weeks or less in the majority of patients. Two patients who stopped interferon treatment given flu-like symptoms and intolerable rash, respectively, showed rapid recurrence of CME.Conclusions: Weekly administration of pegylated interferon alfa-2A is an effective treatment for refractory inflammatory CME, though side effects may limit tolerability in some patients.

An Update on Endocrine Mucin-producing Sweat Gland Carcinoma: Clinicopathologic Study of 63 Cases and Comparative Analysis.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm with predilection for the periorbital skin of older women. Histologically and immunophenotypically, EMPSGC is analogous to another neoplasm with neuroendocrine differentiation, solid papillary carcinoma of the breast. Both lesions are spatially associated with neuroendocrine mucinous adenocarcinomas of the skin and breast, respectively. EMPSGC is ostensibly a precursor of neuroendocrine-type mucinous sweat gland adenocarcinoma (MSC), a lesion of uncertain prognosis. Non-neuroendocrine MSC has been deemed locally aggressive with metastatic potential, and previous works speculated that EMPSGC-associated (neuroendocrine-type) MSC had similar recurrence and metastatic potential with implications for patient follow-up. Only 96 cases of EMPSGC have been reported (12 cases in the largest case series). Herein, we present 63 cases diagnosed as "EMPSGC" in comparison with aggregated results from known published EMPSGC cases. We aim to clarify the clinicopathologic features and prognostic significance of the neuroendocrine differentiation of EMPSGC and its associated adenocarcinoma and to determine the nosological relevance of EMPSGC association in the spectrum of MSC histopathogenesis. Results established an overall female predominance (66.7%) and average presenting age of 64 years. EMPSGC lesions were associated with adjacent MSC in 33.3% of cases. The recurrence rate for neuroendocrine-type MSC was ~21%, less than the reported 30% for non-neuroendocrine MSC. There were no cases of metastasis. EMPSGC and neuroendocrine-type MSC are distinct entities with more indolent behavior than previously reported, supporting a favorable prognosis for patients.

Orbital Cellular Epithelioid Hemangioma.

PURPOSE: To report a case of orbital cellular epithelioid hemangioma (EH) in which FOSB and CAMTA1 immunostains were used to detect a cytogenetic rearrangement as an adjunctive tool in diagnosis. METHODS: Case report. RESULTS: A patient with a history of prior ligation of a presumed orbital varix presented with recurrent proptosis. Imaging revealed a highly vascular right orbital mass. Microscopic examination revealed a circumscribed neoplasm composed of plump epithelioid endothelial cells with copious mildly eosinophilic cytoplasm and relatively uniform vesicular nuclei. To aid in diagnosis, immunostains for FOSB and CAMTA1 were performed to detect corresponding cytogenetic rearrangements. The presence of multifocal nuclear positivity for FOSB, indicating FOSB genetic rearrangement, and negativity for CAMTA1 were considered reassuring features against a diagnosis of a malignant epithelioid hemangioendothelioma (EHE), supporting a diagnosis of benign cellular EH. CONCLUSIONS: This case report demonstrates that the use of immunohistochemical stains to detect cytogenetic rearrangements may aid in the distinction between benign EH and malignant EHE. It also reminds providers of the clinical and histopathologic features of this lesion, which occurs rarely in the orbit, and helps clarify the evolving nomenclature surrounding epithelioid hemangioma.

Intraoperative Optical Coherence Tomography to Guide Corneal Biopsy: A Case Report.

PURPOSE: To report on a new application of intraoperative optical coherence tomography (OCT) as a real-time imaging tool to help guide corneal biopsy. METHODS: Single case report. After the negative result of a corneal biopsy, we used intraoperative OCT during repeat biopsy to assist in depth and acquisition of an anterior stromal opacity suspected to be immunoglobulin deposits. RESULTS: The use of intraoperative OCT is a novel tool, which assisted in successful identification and conservative biopsy of corneal immunoglobulin deposits. CONCLUSIONS: Intraoperative OCT is a novel tool that assists in the identification and location of corneal pathology for biopsy.

Characterization of corneal deposition keratopathy in the setting of blood cell dyscrasia and a minimally invasive technique to clear the cornea in a single case.

PURPOSE: To report 3 cases of corneal protein deposition occurring in association with systemic disease, with one case demonstrating a novel technique for clearing corneal deposits. OBSERVATIONS: Three patients presented with corneal deposits associated with systemic disease. Corneal involvement was noted prior to diagnosis of systemic disease in two patients, leading to subsequent diagnosis of multiple myeloma or monoclonal gammopathy of undetermined significance. OCT revealed protein deposition at various corneal levels, including at different stromal depths in the two cases of multiple myeloma. A novel technique of posterior endothelial scraping was performed in one case with deep stromal deposits, leading to significant corneal clearing. CONCLUSIONS AND IMPORTANCE: This case series demonstrates that recognition of corneal deposits may precede the diagnosis of systemic disease. It incorporates the use of anterior segment OCT to characterize corneal deposits, and demonstrates a novel surgical technique for clearing certain types corneal deposits.