皮肤Merkel细胞癌4例临床病理分析

Merkel cell carcinoma: a clinicopathologic analysis of four cases

目的探讨皮肤Merkel细胞癌的临床病理特征及预后。方法收集4例皮肤Merkel细胞癌分析其临床表现、病理特征及免疫表型,并电话随访及文献复习。结果4例患者男性2例,女性2例,发病年龄67~80岁,中位年龄75岁。发病部位分别为小腿、上臂、手腕和臀部。均行肿物扩大切除术,其中2例行术后放疗和化疗。镜下见肿瘤累及真皮及皮下纤维、脂肪组织。免疫组化染色显示上皮性标记物CKpan和EMA(+),CK20(+)并具有特异性的核周点状着色或胞质弥漫着色;神经内分泌标记物Syn、CgA和CD56(+)。4例均获得随访,随访时间5~29月。1例于术后27个月因肿瘤全身多处转移死亡,余3例存活。结论皮肤Merkel细胞癌较罕见,有复发转移倾向,形态学特点及免疫组化结果有助于诊断,治疗推荐行扩大手术切除术并行前哨淋巴结活检,术后辅以放化疗。

Objective To explore the clinicopathological features and prognosis of Merkel cell carcinoma. Methods Four cases of Merkel cell carcinoma were collected for clinical pathology analysis and immunohistochemical staining,with telephone follow-up and literature review. Results This group consisted of 2 males and 2 females,aged 67 to 80 years (median age 75 years). The location of the disease was the calf,upper arm,wrist and buttocks. All patients underwent extended mass resection and 2 patients underwent postoperative radiotherapy and chemotherapy. Microscopically,the tumor infiltrated the dermis and subcutaneous tissue. Immunohistochemical staining showed that CKpan and EMA were positive,CK20 had specific perinuclear dot-like staining or cytoplasmic diffuse staining,and neuroendocrine markers Syn,CgA and CD56 were positive. The follow-up data were available in 4 cases,one patient died of multiple metastases in the tumor at 27 months after surgery,and other 3 patients were still alive at 5 to 29 months. Conclusions Skin Merkel cell carcinoma is rare,with recurrence and metastasis tendency. Morphological features and immunohistochemistry are helpful for diagnosis.The recommended treatment is extended surgical resection and sentinel lymph node biopsy,followed by radiotherapy and chemotherapy.