肺母细胞瘤临床病理特征分析

Clinicopathological characteristics and differential diagnosis on pulmonary blastoma

关键词:  肺母细胞瘤  临床病理学  诊断

Key words:  [Key words]　pulmonary blastoma  clinicopathology  diagnosis

摘要:

[摘要]　目的　探讨肺母细胞瘤的临床病理特征。 　方法　回顾性分析1987年1月至2013年9月8例肺母细胞瘤的临床病理资料，8例肺母细胞瘤中成人占6例，其中经典的双相型肺母细胞瘤4例，由原始上皮成分和原始间叶成分共同组成；上皮型肺母细胞瘤2例，由分化较好的形态类似于子宫内膜样的腺体构成。其它2例为小儿胸膜肺母细胞瘤，由恶性胚胎性间叶成分构成。对肿瘤组织进行免疫组化染色及EGFR基因突变检测。 　结果　免疫组化染色显示原始上皮成分广谱CK、EMA、TTF1、β-catenin阳性，原始间叶成分Vimentin阳性，桑葚体结构CgA和Syn阳性。所有病例EGFR基因突变检测显示EGFR基因18～21号外显子无突变，均为野生型。　结论　肺母细胞瘤是罕见的肺原发性恶性肿瘤，免疫组化染色有助于明确诊断。

Abstract:

[Abstract] Objective To investigate clinicopathological characteristics and differential diagnosis of pulmonary blastoma(PB). Methods The clinical pathological data of 8 patients from Jan. 1987 to Sep. 2013 were reviewed retrospectively. Six cases were adults with the age range of 27-53 (mean 42). Four of them were classic biphasic pulmonary blastoma(CBPB) which were composed both of the primitive epithelial and stromal components. Two of them were epithelial type pulmonary blastoma which was composed of well differentiated tubules resembling endometrioid gland. The other two cases were children with pleuropulmonary blastoma which was composed of malignant embryonic stroma and non-neoplastic epithelium. CK, EMA, Nse, CgA, Syn, Vimentin, SMA, Des, S100, CD34, TTF1,β-catenin were detected by immunohistochemistry and EGFR mutation in exon 18-21 was detected by ARMS. Results The pan-CK, EMA, TTF1 and β-catenin were positive in the epithelial component, vimentin positive in the stromal component, and CgA and Syn was positive in the structure of morula. EGFR mutation in exon 18-21 were not detected in all the 8 cases. Conclusion Pulmonary blastoma is a rare primary lung malignancy. The immunohistochemical staining is helpful for its diagnosis.