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[默克尔细胞癌]

[Merkel cell carcinoma].

作者信息

Verola O, Champeau F

机构信息

Service d'Anatomie et de Cytologie Pathologique, Hôpital Saint-Louis, Paris, France.

出版信息

Ann Chir Plast Esthet. 1998 Aug;43(4):439-44.

PMID:9926475
Abstract

Merkel cell carcinoma (MCC) is a malignant cutaneous neuroendocrine tumor which may be difficult to diagnose. It mostly occurs in old patients and the commonest sites are the skin of the head and neck and the extremities. MCC appears as a solitary violaceous dome-shaped nodule or indurated plaque. Histopathologic diagnosis may be difficult and three main patterns have been described. With immunohistochemistry studies, MCC express both epithelial (cytokeratins, EMA) and neuroendocrine (NSE, chromogranin, ...) markers. The tumor develops an aggressive course not unlike an aggressive melanoma. Local recurrence and regional lymph node metastases occur in 40 to 75% of cases. Long-term prognosis is unfavorable (3-year survival rate is 55%). Wide surgical excision associated with radiotherapy is the treatment of choice, regional lymph node metastases should be treated by lymph node excision and radiotherapy; chemotherapy should be used in systemic disease.

摘要

默克尔细胞癌(MCC)是一种恶性皮肤神经内分泌肿瘤,可能难以诊断。它主要发生于老年患者,最常见的部位是头颈部皮肤和四肢。MCC表现为单个紫蓝色圆顶状结节或硬结性斑块。组织病理学诊断可能困难,已描述了三种主要模式。通过免疫组织化学研究,MCC表达上皮(细胞角蛋白、上皮膜抗原)和神经内分泌(神经元特异性烯醇化酶、嗜铬粒蛋白等)标志物。该肿瘤发展进程侵袭性强,与侵袭性黑色素瘤无异。40%至75%的病例会出现局部复发和区域淋巴结转移。长期预后不佳(3年生存率为55%)。广泛手术切除联合放疗是首选治疗方法,区域淋巴结转移应通过淋巴结切除和放疗进行治疗;全身性疾病应使用化疗。

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