【摘要】 目的:探讨皮肤Merkel细胞癌(Merkel cell carcinoma, MCC)的临床病理特征、诊断及鉴别诊断、治疗及预后。方法:回顾性分析5例皮肤MCC的临床资料，观察组织学形态及免疫表型，讨论鉴别诊断，总结治疗和预后进展。结果:5例患者中男性3例、女性2例，中位年龄为71岁。2例肿瘤位于头面部、其余3例位于臀部、前臂和腹股沟。肿瘤最大径为1.8 cm～6.1 cm,边界较清，无包膜，切面灰白，质中。镜下见肿瘤位于真皮，表皮无累及，肿瘤细胞排列成巢团状、弥漫状或片状。肿瘤细胞中等偏小，圆形或椭圆形，胞质少，部分可见核仁。个别病例细胞异型性较大，核分裂象丰富。免疫组化染色结果示：CK-Pan、CK20、EMA、CgA、Syn及CD56阳性表达，核抗原Ki-67阳性表达指数为35%～90%;vimentin、CEA、CD99、TTF-1及LCA阴性表达。发生于腹股沟的病例于术后5个月死亡，发生于前臂和头面部的3个病例于术后18～24月死亡，发生于臀部的病例随访3个月仍无瘤生存。结论:皮肤MCC常见于老年患者，好发部位为头面部及四肢躯干。肿瘤细胞形态多样，结构差异大，对原发于皮肤的恶性肿瘤的诊断及鉴别诊断时应考虑该肿瘤。MCC是高度侵袭性神经内分泌肿瘤，容易发生复发及转移，预后较差。扩大切除肿瘤及淋巴结清扫并辅助以放化疗是目前建议的治疗方式。更多还原

【Abstract】 Objective:To explore clinicopathologic characteristic, diagnosis and differential diagnosis, therapy and prognosis of cutaneous merkel cell carcinoma(MCC).Methods:Clinical data on 5 cases of cutaneous MCC were retrospectively analyzed.Histopathologic change and immunophenotype were observed.Diagnosis and differential diagnosis were discussed.Advancement of treatment and prognosis were summarized.Results:There were 3 males and 2 females in five patients.The median age of the 5 patients was 71 years old.2 cases of MCC located in head and face, the other 3 cases located in gluteal region, forearm and groin respectively.Tumor sizes were variant from 1.8 cm to 6.1 cm.These masses with distinct margin were without capsule and with a grayish-white cross section.The tumors had a moderate quality.Under microscopic study, tumors located in dermis, but not involved the epidermis.Tumor cells arranged with nest, diffuse and lamellar pattern.The round or oval tumor cells were with moderate or rather small size and small amounts of cytoplasm.Nucleoli could be seen in part of the tumor cells and cellular heteromorphism was obvious in individual case with rich mitosis.Immunohistochemistry staining result showed that the tumor cells were positive for CK-Pan, CK20,EMA,CgA,Syn and CD56,but negative for vimentin, CEA,CD99,TTF-1 and LCA.The positive indexes of Ki-67 were variant from 35%～90%.One patient with tumor in gluteal region was dead in 5 months postoperatively and the other 3 patients with tumors in forearm, head and face passed in 18～24 months after operation.The remaining one with tumor in groin was alive without evidence of disease for 3 months.Conclusion:MCC is common in old age patients and often involve head, face and limbs.The tumor cells present with a variety of shapes and is arrange with variable structures.MCC should be considered when diagnosis or differential diagnosis are made for a primary cutaneous malignant tumor.MCC is a kind of highly aggressive endocrine tumor and easy to be recurrence and metastasis.According to poor prognosis of MCC,wide margin surgical excision, lymph node dissection and adjuvant radiotherapy and chemotherapy are recommend for ideal treatment.更多还原