CAJ | 학술논문

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。
목적탐토폐모세포류적림상병리특정。방법회고성분석1987년1월지2013년9월8례폐모세포류적림상병리자료，8례폐모세포류중성인점6례，기중경전적쌍상형폐모세포류4례，유원시상피성분화원시간협성분공동조성；상피형폐모세포류2례，유분화교호적형태유사우자궁내막양적선체구성。기타2례위소인흉막폐모세포류，유악성배태성간협성분구성。대종류조직진행면역조화염색급EGFR기인돌변검측。결과면역조화염색현시원시상피성분엄보CK、EMA、TTF1、β－catenin양성，원시간협성분Vimentin양성，상심체결구CgA화Syn양성。소유병례EGFR기인돌변검측현시EGFR기인18～21호외현자무돌변，균위야생형。결론폐모세포류시한견적폐원발성악성종류，면역조화염색유조우명학진단。
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 epi-thelium.CK, EMA, Nse, CgA, Syn, Vimentin, SMA, Des, S100, CD34, TTF1,β-catenin were detected by immuno-histochemistry and EGFR mutation in exon 18-21 was detected by ARMS.Results The pan-CK, EMA, TTF1 andβ-catenin were positive in the epithelial component, vimentin positive in the stromal component, and CgA and Syn was posi-tive 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.