Gyoo Huh
Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Korea.
Title: A Case of Endocrine Mucin-producing Sweat Gland Carcinoma associated Neuroendocrine Type Primary Cutaneous Mucinous Carcinoma
Biography
Biography: Gyoo Huh
Abstract
Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal carcinoma that most commonly presenting on the periorbital skin of elderly women. It was first reported by Flieder et al in 1997 and is recently proposed as an in situ precursor of mucinous carcinoma with neuroendocrine features. EMPSGC and associated mucinous carcinoma likely to behave more indolently than non-endocrine subtype.
A 62-year-old male presented with a year history of an asymptomatic, skin-colored nodule on the left cheek near the edge of lower eyelid. Based on the clinical impression of intradermal nevus, a punch excisional biopsy was performed. Histopathologic findings demonstrated well-circumscribed, dermal nodule comprising bland, uniform tumor cells with stippled chromatin. Also, floating tumor nests were demonstrated within abundant mucin pool. Staining for p63 revealed stromal myoepithelial cells. Immunohistochemistry stains were positive for CK7, CD56, synaptophysin, and chromogranin A, rendering a diagnosis of EMPSGC-associated neuroendocrine primary cutaneous mucinous carcinoma (PCMC). Owing to unclear resection margin, the patient is scheduled to undergo wide excision.
Although rare, dermatologists must be aware of EMPSGC-associated neuroendocrine PCMC as these tumors can be locally destructive and cosmetically disfiguring. To our knowledge, this is the first report in the Korean literature.