A case of endocrine mucin-producing sweat gland carcinoma

Department of Dermatology, University of Yamanashi, Yamanashi, Japan.
Pathology International (Impact Factor: 1.69). 05/2012; 62(5):344-6. DOI: 10.1111/j.1440-1827.2012.02793.x
Source: PubMed


Endocrine mucin-producing sweat gland carcinoma (EMPSGC), which is an uncommon sweat gland tumor with a predilection for the eyelids, is morphologically analogous to solid papillary carcinoma of the breast. We report the case of a 55-year-old man with a subcutaneous tumor of the upper cheek. The pathological findings for this patient were compatible with those of reported cases of EMPSGC, and p63 staining revealed partial microinvasion into the dermis. On the basis of these findings, the patient was diagnosed with EMPSGC. It is reported that EMPSGC is a precursor of invasive mucinous carcinoma of the skin. Therefore, this patient was treated and followed up as if he had mucinous carcinoma of the skin. To the best of our knowledge, this is the first report of such a case from Japan.

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    ABSTRACT: PURPOSE: To describe the clinical and pathologic characteristics of mucin-producing sweat gland carcinoma of the eyelid and to determine whether neuroendocrine differentiation is of prognostic significance. DESIGN: Retrospective interventional case series. METHODS: Search of the New York Eye and Ear Infirmary pathology database between 1990 and 2011 identified 16 patients with mucin-producing sweat gland carcinoma. Clinical, histopathologic, and immunohistochemical analyses were performed on all identified cases. RESULTS: The patients presented with vascularized, focally cystic, nonulcerated eyelid margin lesions. Histopathologic evaluation showed that 4 lesions (25%) had a cystic, papillary, and solid growth pattern with an in situ component, 7 (44%) were pure invasive mucinous carcinomas, and 5 (31%) demonstrated both growth patterns. Immunohistochemical analysis of 15 tumors showed that pure cystic/papillary lesions had a significantly greater percentage of synaptophysin-immunoreactive cells (P = .036). There was no significant difference in the number of neuroendocrine markers expressed or in the intensity of immunostaining among the 3 different growth patterns. Re-excision for margin clearance was performed in 8 of 13 cases (61.5%). Two of 13 lesions recurred (15%); 1 of these was an in situ tumor with cystic morphology and neuroendocrine differentiation and the other was pure invasive mucinous carcinoma. None of the lesions metastasized. CONCLUSIONS: Mucin-producing sweat gland carcinoma pathologically represents a continuum, from an in situ lesion to a classic, invasive mucinous carcinoma. Immunohistochemical evidence of neuroendocrine differentiation can be observed in all lesions and does not appear to have a prognostic significance, arguing against the utility of immunohistochemical subtyping of mucinous sweat gland carcinomas.
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    ABSTRACT: Endocrine mucin-producing sweat gland carcinoma (EMPS) is a rare low-grade sweat gland carcinoma with an infiltrating growth pattern. It occurs mostly in women and shows a predilection for the periorbital region. Histopathologically, the tumor shows analogous features to endocrine ductal carcinoma/solid papillary carcinoma of the breast and shares some clinical and morphological similarities with primary mucinous carcinoma of the skin. The tumor is characterized by large monomorphous epithelial cells with little nuclear pleomorphism and only a few mitotic figures. The solid cystic tumor shows mucin-filled small cystic spaces, cribriform areas and expresses the neuroendocrine markers synaptophysin, chromogranin and neuron-specific enolase with varying staining intensities. The tumor cells are also positive for estrogen and progesterone receptors. We present three cases of this rare tumor with typical clinical, histopathological and immunohistochemical findings, give a short summary of the literature and discuss the most relevant differential diagnoses.
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