Acebo E, Vidaurrazaga N, Varas C, Burgos-Bretones J J, Díaz-Pérez J L
Department of Dermatology, Hospital de Cruces, Barcaldo, Vizcaya, Spain.
J Eur Acad Dermatol Venereol. 2005 Sep;19(5):546-51. doi: 10.1111/j.1468-3083.2005.01224.x.
To report our 12-year experience with Merkel cell carcinomas (MCCs) from a clinical and pathological point of view.
Eleven MCCs were diagnosed at our institution between 1991 and 2002.
A retrospective clinical, histopathological and immunohistochemical study was performed. Age, gender, location, size, stage, treatment and follow-up data were collected. Histopathological pattern and immunohistochemical study with CAM 5.2, cytokeratin 20 (CK20), CK7, Ber EP4, neurofilaments, synaptophysin, chromogranin, S100 protein, p53 protein, CD117, leucocyte common antigen (LCA) and Ki-67 were accomplished.
Six females and five males with a mean age of 82 years were identified. Tumours were located on the face (n = 6), extremities (n = 3) and trunk (n = 1). At diagnosis, one patient was in stage Ia, six in stage Ib, three in stage II and one in stage III. All but one patient experienced wide surgical excision of the tumour. Additional treatment consisted of lymph node dissection in two patients, radiotherapy in four patients and systemic chemotherapy in one patient. Local recurrence developed in five patients. Three patients died because of MCC after 14 months of follow-up. Intermediate-size round cell proliferation was found in all cases. Additional small-size cell pattern and trabecular pattern were observed in seven and six cases, respectively. Eccrine and squamous cell differentiation were found in three cases. A dot-like paranuclear pattern was observed in all cases with CAM 5.2 and neurofilaments, and in 89% of cases with CK20. Seventy-five per cent of cases reacted with Ber EP4, chromogranin and synaptophysin, 70% with p53, 22% with S100 protein, 55% with CD117 and none with LCA. Ki-67 was found in 75% of tumoral cells on average. Fifty per cent of MCCs reacted with CK7 and showed eccrine differentiation areas.
MCC is an aggressive neuroendocrine tumour of the elderly. Wide surgical excision is the recommended treatment. Lymph node dissection, adjuvant radiotherapy and chemotherapy decrease regional recurrences but have not been demonstrated to increase survival. Immunohistochemically, MCC is an epithelial tumour with neuroendocrine features.
从临床和病理角度报告我们12年来对默克尔细胞癌(MCC)的诊治经验。
1991年至2002年期间,我院共诊断出11例MCC。
进行回顾性临床、组织病理学和免疫组化研究。收集年龄、性别、部位、大小、分期、治疗及随访数据。完成组织病理学模式及采用CAM 5.2、细胞角蛋白20(CK20)、CK7、Ber EP4、神经丝、突触素、嗜铬粒蛋白、S100蛋白、p53蛋白、CD117、白细胞共同抗原(LCA)和Ki-67进行免疫组化研究。
共确定6例女性和5例男性,平均年龄82岁。肿瘤位于面部(n = 6)、四肢(n = 3)和躯干(n = 1)。诊断时,1例患者为Ia期,6例为Ib期,3例为II期,1例为III期。除1例患者外,所有患者均接受了肿瘤广泛手术切除。另外的治疗包括2例患者行淋巴结清扫,4例患者行放疗,1例患者行全身化疗。5例患者出现局部复发。3例患者在随访14个月后因MCC死亡。所有病例均发现中等大小的圆形细胞增殖。另外,分别在7例和6例中观察到小细胞模式和小梁模式。3例出现汗腺和鳞状细胞分化。在所有使用CAM 5.2和神经丝的病例中以及89%使用CK20的病例中观察到点状核旁模式。75%的病例与Ber EP4、嗜铬粒蛋白和突触素反应,70%与p53反应,22%与S100蛋白反应,55%与CD117反应,无一例与LCA反应。平均75%的肿瘤细胞中发现Ki-67。50%的MCC与CK7反应并显示汗腺分化区域。
MCC是一种侵袭性的老年神经内分泌肿瘤。推荐采用广泛手术切除治疗。淋巴结清扫、辅助放疗和化疗可减少局部复发,但尚未证明能提高生存率。免疫组化方面,MCC是一种具有神经内分泌特征的上皮性肿瘤。