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November 15, 2010

Pathology Quiz Case 2: Diagnosis

Author Affiliations

Copyright 2010 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2010

Arch Otolaryngol Head Neck Surg. 2010;136(11):1148-1149. doi:10.1001/archoto.2010.199-b

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