Chen S W, Chang S T, Ho C H, Wang J S, Wang R C, Takeuchi K, Chuang S S
Lioying Chi-Mei Hospital, Department of Internal Medicine, Division of Hemato-Oncology, Tainan, Taiwan.
Lioying Chi-Mei Hospital, Department of Pathology, Tainan, Taiwan.
Malays J Pathol. 2022 Apr;44(1):61-66.
Merkel cell carcinoma (MCC) is a rare malignant cutaneous neuroendocrine tumour affecting mainly elderly patients and is more common in the West than in Asia. It is associated with Merkel cell polyomavirus (MCPyV), immunosuppression, and ultraviolet light. In this study, we retrospectively investigated the first series of MCC from Taiwan and identified 19 cases from three tertiary centres. All patients were males with a median age of 67.5. Twelve (63%) cases occurred in the extremities, with one unique case presenting initially as nodal metastasis of unknown primary. Immunohistochemically, the great majority of tumours expressed CK20 (89%), synaptophysin (89%), and INSM1 (84%), with none positive for TTF1. Eleven (58%) cases were positive for MCPyV by immunohistochemistry (clone CM2B4). All patients were treated with excision, including four with additional radiotherapy and one with radiotherapy and chemotherapy. Nodal status and treatment modalities significantly affected survival. The median survival time of MCPyV-positive cases was much longer than the negative cases (median 40 vs. 10 months). In summary, we presented the first report on the clinicopathological features of MCC in Taiwan, with 58% cases associated with MCPyV. The prognosis of patients with MCPyV-positive tumours was better than those negative for MCPyV.
默克尔细胞癌(MCC)是一种罕见的皮肤恶性神经内分泌肿瘤,主要影响老年患者,在西方比在亚洲更常见。它与默克尔细胞多瘤病毒(MCPyV)、免疫抑制和紫外线有关。在本研究中,我们回顾性调查了台湾的首批MCC病例系列,并从三个三级中心确定了19例病例。所有患者均为男性,中位年龄为67.5岁。12例(63%)发生在四肢,其中1例独特病例最初表现为原发灶不明的淋巴结转移。免疫组化显示,绝大多数肿瘤表达细胞角蛋白20(CK20,89%)、突触素(89%)和胰岛1号转录因子(INSM1,84%),无一例TTF1阳性。11例(58%)病例免疫组化检测MCPyV(克隆号CM2B4)呈阳性。所有患者均接受了手术切除治疗,其中4例接受了额外的放疗,1例接受了放疗和化疗。淋巴结状态和治疗方式显著影响生存。MCPyV阳性病例的中位生存时间比阴性病例长得多(中位时间分别为40个月和10个月)。总之,我们首次报告了台湾MCC的临床病理特征,58%的病例与MCPyV有关。MCPyV阳性肿瘤患者的预后优于MCPyV阴性患者。