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Endokrines muzinproduzierendes Schweißdrüsenkarzinom

Drei Fallberichte mit kurzer Literaturübersicht

Endocrine mucin-producing sweat gland carcinoma

Three case reports with a brief review of the literature

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Zusammenfassung

Das endokrine muzinproduzierende Schweißdrüsenkarzinom (EMPS) ist ein seltenes, niedrigmalignes, lokal infiltrierendes Schweißdrüsenkarzinom. Es tritt überwiegend bei Frauen in der Periorbitalregion auf. Histopathologisch weist der Tumor Analogien zum endokrinen duktalen Carcinoma in situ bzw. zum solide-papillären Karzinom der Brustdrüse auf. Der Tumor hat klinisch und feingeweblich Ähnlichkeiten mit dem primären muzinösen Karzinom der Haut. Das EMPS ist charakterisiert durch große epitheliale Zellen mit geringen Kernatypien und wenigen Mitosen. Die solide-zystischen Tumorverbände enthalten mit Muzin gefüllte zystische Strukturen und kribriforme Abschnitte. Sie sind immunhistochemisch in wechselnder Intensität markierbar mit den neuroendokrinen Markern Synaptophysin, Chromogranin und NSE. Die Zellkerne des Tumors sind Östrogen- und Progesteronrezeptor-positiv. Anhand von 3 Fällen stellen wir die typischen klinischen, histologischen und immunhistochemischen Befunde dieses seltenen Tumors dar und geben einen kurzen Überblick über die aktuelle Literatur und die wichtigsten Differenzialdiagnosen.

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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Danksagung

Wir danken Frau Prof. Reiner und Herrn Prof. Grillenberger vom Pathologisch-Bakteriologischen Labor am Donauspital, Wien, für das Blockmaterial und die klinischen Daten des zweiten Falls.

Einhaltung ethischer Richtlinien

Interessenkonflikt. A. Rütten, T. Mentzel und L. Requena geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Dermatopathologie Bodensee, Siemensstr. 6/1, 88048, Friedrichshafen, Deutschland

    A. Rütten & T. Mentzel

  2. Department of Dermatology, Fundacion Jimenez Diaz, Madrid, Spanien

    L. Requena

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  1. A. Rütten
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  2. T. Mentzel
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  3. L. Requena
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Correspondence to A. Rütten.

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Rütten, A., Mentzel, T. & Requena, L. Endokrines muzinproduzierendes Schweißdrüsenkarzinom. Pathologe 35, 462–466 (2014). https://doi.org/10.1007/s00292-014-1936-9

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  • DOI: https://doi.org/10.1007/s00292-014-1936-9

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