原发性皮肤黏液癌2例临床病理观察

目的探讨原发性皮肤黏液癌的临床病理特征、诊断及鉴别诊断、治疗及预后。方法回顾性分析2例原发性皮肤黏液癌的临床病理特征、免疫表型,对患者进行随访,并复习相关文献总结其临床和病理组织学特征。结果2例均为男性,年龄分别为62岁及47岁。临床以面部和眼角无痛性肿块就诊。大体均为皮下边界尚清的灰白质韧结节,切面灰白呈胶冻样;镜下肿瘤多位于真皮层,可累及皮下脂肪组织,境界清楚,无包膜,肿瘤背景为大量黏蛋白形成黏液湖,并被纤细的纤维间隔分割成多房状或蜂窝状,其中漂浮着大小不一的细胞巢,肿瘤上皮团呈条索状、腺样、筛孔状、乳头状或微乳头状排列,细胞巢团间血管稀少或缺如,细胞圆形或多方形,胞质嗜酸性,个别细胞内含黏液;核轻度异形,染色质细腻,可见小核仁,核分裂象少见;免疫表型:肿瘤细胞角蛋白CAM5.2、CK7、上皮膜抗原(epithelial membrane antigen,EMA)、癌胚抗原(carcino-embryonic antigen,CEA)均(+),但CK20(-);GATA-3弥漫(+),ER、PR呈弥漫核强(+),AR、GCDFP-l5、S-100不同程度(+);Syn、Cg A均为(-);肿瘤细胞Ki-67增殖指数5%~10%。结论原发性皮肤黏液癌较罕见,多见于老年男性,临床表现无特异性易误诊,组织学上需与多部位转移性黏液癌鉴别,肿瘤易复发,少见转移。

Primary cutaneous mucinous carcinoma:a clinicopathological analysis of two cases and review of literature

Purpose To investigate the clinicopathological features,diagnosis,differential diagnosis,treatment and prognosis of primary cutaneous mucinous carcinoma.Methods The clinicopathological features and immunophenotype of2 cases of primary cutaneous mucinous carcinoma were analyzed retrospectively.The patients were followed up and the clinical and histopathological features were summarized by reviewing the relevant literature.Results The two cases were all male,aged 62 and 47 years respectively.The initial symptom was a painless mass in the face and corner of the eye.The gross specimens showed that the two cases were gray white tough nodules with clear subcutaneous boundary,and the cutting section was gray and gelatinous.Microscopically,most tumors were located in the dermis,and invaded the subcutaneous adipose tissue,with a clear boundary and no capsule.A large number of mucins formed mucinous lakes,which were divided into multiple chambers or honeycombs by the delicate fiber.There were cell nests of different sizes floating on them.The tumor epithelial masses were arranged in the form of cords,glandular,sieve,papillary or micropapillary.There were few or absent blood vessels between cell nests.The cells were round or square in shape,eosinophilic in cytoplasm and mucus in individual cells;the nuclei were slightly heteromorphic and finely stained,with small nucleoli and rare mitosis.Immunohistochemistry showed tumor cytokeratin CAM5.2,CK7,epithelial membrane antigen(EMA)and carcinoembryonic antigen(CEA)were all positive,but CK20 was negative.GATA-3 was diffuse positive.ER and PR were diffuse nuclear strong positive.AR,GCDFP-l5 and S-100 were positive in different degree.SYN and CGA were all negative.Ki-67 proliferation index of tumor cells was 5%~10%.Conclusion Primary cutaneous mucinous carcinoma is rare and commonly occurs in elderly men.It is easy to be misdiagnosed without specific clinical manifestations.Histologically,it needs to be differentiated from multi site metastatic mucinous carcinoma.The tumor is easy to recur and rarely metastasized.