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目的探讨母细胞性浆细胞样树突细胞肿瘤(BPDCN)临床病理特征。方法收集6例BPDCN患者临床信息、病理资料;所有病例均行HE、免疫组化染色和EBV-EBER原位杂交。结果男性5例,女性1例,平均年龄45.2岁。均表现为皮肤肿瘤。组织学改变为真皮全层至皮下脂肪组织见肿瘤细胞结节状或弥散浸润,表皮未受累;细胞中等大小,较一致,核质比高,核圆形或不规则,染色质细腻,核分裂象多见。免疫表型6例肿瘤细胞CD4和CD123均(+),5例CD7和CD56(+),2例Td T弥漫(+),2例CD68(+),1例CD117灶性(+),Ki-67阳性范围30%~80%,CK、EMA、CD3、CD20、CD30、CD34、S-100、MPO(-);EBV-EBER均为(-)。结论 BPDCN是罕见的淋巴造血系统肿瘤,好发于皮肤,诊断需结合临床、形态学与免疫组化。
Objective To investigate the clinicopathological features of bcllc cytoplasmic dendritic cell tumor (BPDCN). Methods The clinical data and pathological data of 6 patients with BPDCN were collected. HE, immunohistochemical staining and EBV-EBER in situ hybridization were performed in all cases. Results There were 5 males and 1 females, with an average age of 45.2 years. All showed skin tumors. Histological changes to full-thickness dermis to subcutaneous adipose tissue see tumor cells nodular or diffuse infiltration, epidermis is not involved; cells of medium size, more consistent, high nuclear mass, nuclear round or irregular, fine chromatin, mitotic More common. The immunophenotypes included 6 cases of CD4 and CD123 (+), 5 cases of CD7 and CD56 (+), 2 cases of TdT diffuse (+), 2 cases of CD68 (+), 1 case of CD117 foci -67 positive range of 30% to 80%, CK, EMA, CD3, CD20, CD30, CD34, S-100, MPO; Conclusion BPDCN is a rare tumor of lymphatic hematopoietic system and occurs in the skin. Diagnosis should be combined with clinical, morphological and immunohistochemical findings.