Endocrine Mucin-Producing Sweat Gland Carcinoma, a Histological Challenge

Abstract

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare adnexal tumor of the skin with low-grade cytological features and neuroendocrine differentiation. It has a predilection for the skin of the eyelid, but has also been reported in the face and rarely extra-facial locations. The tumor is seen more frequently in women and on average affects the elderly. It is histologically and immunohistochemically analogous to solid papillary carcinoma of the breast/endocrine ductal carcinoma in situ with a nodular, solid, papillary, and/or cribriforming architecture, neuroendocrine differentiation, and mucin production. Since it was first described by Flieder et al. in 1997, less than 60 cases have been reported in literature. We describe the morphological and immunohistochemical features of another case with a review of the common histological differential diagnoses and emphasize the salient features that help distinguish this rare neoplasm.

Endocrine mucin‐producing sweat gland carcinoma: Clinicopathologic, immunohistochemical, and molecular analysis of 11 cases with emphasis on MYB immunoexpression

“…Endocrine mucin‐producing sweat gland carcinoma (EMPSGC) is a rare low‐grade primary cutaneous sweat gland carcinoma with predilection for the periorbital skin in elderly female patients. Around 70 cases of EMPSGC have been reported so far (Table ). The tumor exhibits morphologic and immunophenotypical similarity to endocrine ductal carcinoma in situ (DCIS) or solid papillary carcinoma of the breast .…”

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) of the eyelid: Clinicopathologic features, immunohistochemical findings and review of literature

“…EMPSGC co-expresses estrogen and progesterone receptors. [1234567] Variable immunoreactivity for at least 1 neuroendocrine marker is consistent in these tumors. The tumor cells express CK 5/6, CK7, CK8, CK18, CAM5.2, and EMA and are negative for CK20.…”

“…First described in 1997, EMPSGC is rare and usually affects periocular skin of females in their 6 th or 7 th decades. [1234] Rare cases affecting the cheeks, scalp, and extrafacial sites have been described. [456] Zembowicz et al describe the lower eyelid to be more frequently involved as compared to the upper; Hoguet et al report otherwise.…”

“…[12345] EMPSGC may have in-situ and/or invasive patterns. [14] Invasive foci lack discernible myoepithelial cells, but these can be easily demonstrated around tumor nodules in the in-situ lesions and stain positive for p63, calponin and SMA. The 2 patterns may co-exist indicating a progression of the in-situ component to an invasive tumor where nests of tumor cells float in pools of mucin.…”

“…Histpathologically, it is analogous to the mammary solid-papillary carcinoma and considered as being a precursor of cutaneous mucinous carcinoma. [12] It has an indolent clinical course and favorable prognosis. We describe 10 such cases with their clinicopathological and immunohistochemical features and review the existing literature on EMPSGC.…”