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阴囊汗孔癌1例罕见病例并文献复习

An Unusual Case of a Scrotal Porocarcinoma and Review of the Literature.

作者信息

Hartsough Emily Mae, Moran Jakob, Devins Kyle, Wszolek Matthew, Cornejo Kristine M

机构信息

Departments of Pathology, and.

Urology, Massachusetts General Hospital, Boston, MA.

出版信息

Am J Dermatopathol. 2023 Jan 1;45(1):51-55. doi: 10.1097/DAD.0000000000002327. Epub 2022 Nov 2.

Abstract

Porocarcinomas are rare tumors derived from the acrosyringium and eccrine ducts, which most commonly occur on the lower extremities or head and neck region in older adults. Microscopically, they invariably demonstrate continuity with the epithelium, showing downgrowth of broad anastomosing bands with more infiltrative intradermal cords and nests of pleomorphic tumor cells with ductal lumina; an associated poroma may also be seen. We report an unusual case of a porocarcinoma arising on the scrotum of a 55-year-old man. Because of the extraordinary location and the presence of keratinizing squamous differentiation, distinction from a squamous cell carcinoma was particularly challenging. Close examination revealed the presence of a co-existing poroma, and immunohistochemistry revealed loss of YAP1 with diffuse nuclear expression of NUT in both the porocarcinoma and poroma components. This finding is particularly suggestive of a YAP1::NUTM1 fusion which has been reported to be highly specific for poroid neoplasms. Distinction of porocarcinoma from its mimics is important due to the frequent aggressive behavior of this neoplasm.

摘要

汗腺癌是一种罕见的肿瘤,起源于顶泌汗腺导管和小汗腺导管,最常见于老年人的下肢或头颈部区域。在显微镜下,它们总是与上皮组织相连,表现为宽阔的吻合带向下生长,伴有更多浸润性的真皮内条索和具有导管腔的多形性肿瘤细胞巢;也可能见到相关的汗孔瘤。我们报告了一例罕见的发生在一名55岁男性阴囊上的汗腺癌病例。由于其特殊的位置以及存在角化性鳞状分化,与鳞状细胞癌的鉴别特别具有挑战性。仔细检查发现同时存在汗孔瘤,免疫组化显示在汗腺癌和汗孔瘤成分中YAP1缺失,NUT呈弥漫性核表达。这一发现特别提示存在YAP1::NUTM1融合,据报道该融合对汗孔样肿瘤具有高度特异性。由于这种肿瘤常具有侵袭性,因此将汗腺癌与其相似病变区分开来很重要。

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