胸膜肺母细胞瘤临床病理分析  

Pleuropulmonary Blastoma: a Clinicopathological Analysis

作　　者：陈仕高[1,2] 王世凤[1] 高俊[1] 张尚福[1] 

机构地区：[1]四川大学华西医院病理科,成都610041 [2]成都铁路分局医院病理科,成都610081

出　　处：《中国肺癌杂志》2010年第5期550-553,共4页Chinese Journal of Lung Cancer

摘　　要：背景与目的胸膜肺母细胞瘤是一种罕见的且有其独特临床病理特征的恶性肿瘤,本文旨在探讨胸膜肺母细胞瘤的临床病理学特点及鉴别诊断等。方法收集5例胸膜肺母细胞瘤,复习患者临床资料,进行组织学及免疫组化观察,并结合文献复习分析。结果5例胸膜肺母细胞瘤患者年龄为21个月-47个月,平均为32.8个月;肿瘤主要位于胸腔,4例伴有胸腔积液。组织学观察:I型胸膜肺母细胞瘤1例,呈单纯囊性;II型胸膜肺母细胞瘤2例,呈囊实性,实性区伴横纹肌分化及灶性软骨样结节;III型胸膜肺母细胞瘤2例,完全呈实性,可见间变性未分化肉瘤样成分。免疫组化染色显示肿瘤细胞呈Vimentin(+),部分肿瘤细胞呈Desmin和Myogenin(+),软骨样结节呈S-100(+);PCK、EMA、CD99等均(-)。结论胸膜肺母细胞瘤是一种罕见的主要发生于婴幼儿肺和胸膜的高侵袭性恶性肿瘤,各亚型均有其临床病理学特点。诊断方面应与肺先天性囊性腺瘤样畸形、胚胎性横纹肌肉瘤等良恶性病变鉴别。Background and objective Pleuropulmonary blastoma （PPB） is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma. Methods Five cases of PPB were analyzed by light microscopy, immunohistochemis-try and their clinical data, and the relative literatures were reviewed. Results Five cases of patients suffered from PPB were aged from 21 to 47 months （mean 32.8 months）. Most of the masses were located in the thoracic cavities and 4 cases accompanied with pleural effusions. Histologically, these tumors included 1 case of type I PPB which showed pure cystic architecture; 2 cases were type II PPB which showed cystic and solid masses accompanied with rhabdomyoblastic differentiation and nodules of cartilage; the other 2 cases were type III PPB and characterized by absolute solid masses with anaplastic undifferentiated sar-comatous components. Immunohistochemical studies showed that tumor cells were positive for Vimentin and some for Des-min and Myogenin, the nodules of cartilage were positive for S-100. The tumor cells were negative for PCK, EMA and CD99. Conclusion Pleuropulmonary blastoma is a rare and highly aggressive malignancy arising in the lung and pleural of infancy and early childhood. The type I, II and III PPB have unique clinicopathological features respectively. This kind of tumor should be distinguished from some benign and malignant diseases such as congenital cystic adenomatoid malformation （CCAM） and embryonal rhabdomyosarcoma.

关 键 词：胸膜肺母细胞瘤 免疫组化 病理诊断 鉴别诊断 

分 类 号：R734.2[医药卫生—肿瘤]