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默克尔细胞癌合并鲍温病:两例报告,其中一例具有不寻常的免疫表型。

Merkel cell carcinoma concurrent with Bowen's disease: two cases, one with an unusual immunophenotype.

作者信息

Ishida Mitsuaki, Okabe Hidetoshi

机构信息

Division of Diagnostic Pathology, Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Shiga 520-2192, Japan.

出版信息

J Cutan Pathol. 2013 Sep;40(9):839-43. doi: 10.1111/cup.12176. Epub 2013 May 15.

Abstract

The concurrence of Merkel cell carcinoma (MCC) and squamous cell carcinoma (SCC) is well known, and MCC concurrent with Bowen's disease has also been documented. Herein, we describe two cases of MCC concurrent with Bowen's disease, and one case exhibited an unusual immunophenotype. An 86-year-old male (Patient 1) and an 87-year-old female (Patient 2) presented with nodules of the chest and cheek, respectively. Histopathologic study revealed Bowen's disease and a proliferation of small round cells in the dermis and/or subcutis. Immunohistochemically, the round cells expressed endocrine markers. 'Dot' immunopositivity for cytokeratin (CK) (AE1/AE3) was observed in both patients. However, dot-like CK20 positivity was present only in the second tumor, and thyroid transcription factor-1 (TTF-1) was only positive in the first. Both cases were negative for Merkel cell polyomavirus (MCPyV). MCC concurrent with SCC usually does not involve detectable MCPyV infection, which suggests that combined MCC may develop through different tumorigenetic pathways, such as chronic ultraviolet exposure, as compared to pure MCC. Additionally, concurrent tumors may exhibit an unusual immunophenotype, such as TTF-1(+) /CK20((-)) .

摘要

默克尔细胞癌(MCC)与鳞状细胞癌(SCC)同时存在已为人所知,MCC与鲍恩病同时存在也有文献记载。在此,我们描述两例MCC与鲍恩病同时存在的病例,其中一例表现出不寻常的免疫表型。一名86岁男性(患者1)和一名87岁女性(患者2)分别出现胸部和脸颊结节。组织病理学研究显示为鲍恩病以及真皮和/或皮下组织中小圆形细胞的增殖。免疫组织化学检查显示,圆形细胞表达内分泌标志物。两名患者均观察到细胞角蛋白(CK)(AE1/AE3)的“点状”免疫阳性。然而,点状CK20阳性仅出现在第二个肿瘤中,甲状腺转录因子-1(TTF-1)仅在第一个肿瘤中呈阳性。两例均未检测到默克尔细胞多瘤病毒(MCPyV)。MCC与SCC同时存在通常不涉及可检测到的MCPyV感染,这表明与单纯MCC相比,合并的MCC可能通过不同的肿瘤发生途径发展,如长期紫外线暴露。此外,同时存在的肿瘤可能表现出不寻常的免疫表型,如TTF-1(+) /CK20((-)) 。

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