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Christopher Burris, Vasilios Papastefanou, Caroline Thaung, Mandeep Sagoo, Victoria Cohen; Detection of Extrascleral Extension in Uveal Melanoma. Invest. Ophthalmol. Vis. Sci. 2013;54(15):4244.
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Uveal melanoma is the most common primary intraocular malignancy. Extrascleral extension (ESE) by this tumor is rare, but has been associated with an increased rate of orbital recurrence, as well as an overall poor prognosis. Studies looking at clinical detection show low rates when compared with those focusing on histological detection. Due to the prognostic importance of ESE, we seek to compare our clinical, intraoperative, and histological detection rates.
A retrospective cross-sectional case series of eyes enucleated for uveal melanoma was compiled from the admissions records of the London Ocular Oncology Service during the 28-month period between January 2010 and April 2012. Histopathology reports were searched for ESE. If a case was found to have ESE, the surgical, and clinical notes were obtained to determine when it was first diagnosed or suspected (clinically, intraoperatively, or on histological examination). The patients’ ages, genders, treatments prior to enucleation, and adjuvant therapies were recorded. Tumor locations, shapes, sizes, routes of ESE, pigmentation, cellular morphology, and chromosome 3 status were extracted from the clinical notes, histopathologic, and cytogenetic reports when available.
Of 174 eyes enucleated for uveal melanoma, 16 (9%) had histologically proven extrascleral extension. Eight (50%) of these 16 cases were diagnosed clinically, 3 (19%) were discovered intraoperatively, and 5 (31%) were first detected during microscopic examination. 7/7 (100%) of the cases with anterior ESE vs. 1/9 (11%) of cases with posterior ESE were detected clinically.
ESE was clinically undetectable in a significant percentage (50%) of cases, and clinical detection correlated with the location of the extension (anterior or posterior). Although ultrasound is the best clinical modality for detecting posterior ESE, most of our cases were microscopic. This series highlights the importance of a high level of suspicion for ESE in posterior uveal melanoma, as well as good communication between the surgeon and the pathologist.
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