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Pathology Quiz Case 2: Diagnosis

Arch Otolaryngol Head Neck Surg. 2010;136(11):1148-1149. doi:10.1001/archoto.2010.199-b.
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Diagnosis: Primary signet ring carcinoma of the eyelid

Primary signet ring carcinoma of the eyelid is a unique malignant neoplasm that is derived from the sweat glands. It is an extremely rare tumor, with only 23 reported cases, excluding the one described herein.1A variety of names have been used to describe it, including eccrine sweat gland tumor of clear cell origin,2primary mucinous sweat gland carcinoma of the eyelid,3and histiocytoid variant of eccrine sweat gland carcinoma.4The variability in nomenclature reflects the tumor's debated histogenesis, with authors differing in opinion regarding an apocrine or eccrine origin. Despite the differences in terminology, the reported cases share a common morphological appearance, immunohistochemical profile, and clinical behavior. We have chosen to use the term primary signet ring carcinoma of the eyelid, which was recently coined in 2008.1

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The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
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