摘要
目的探讨肺母细胞瘤的临床病理学特点及鉴别诊断.方法收集8例肺母细胞瘤,复习患者病例档案并进行组织学及免疫组化观察和随访.结果8例肺母细胞瘤患者年龄为5～63岁,平均41.6岁;肿瘤主要位于左肺或右肺的上叶,临床分期以T（2～3）、N（2～3）、M（0）为主;术后平均生存11个月.组织学及免疫组化观察：双相性肺母细胞瘤6例,胚胎性腺管样结构显示AE1/AE3（＋）,幼稚的梭形细胞呈vimentin（＋）,软骨分化区S-100（＋）;上皮性肺母细胞瘤1例,桑葚体为特征性结构,免疫组化显示NSE和AE1均（＋）,桑葚体可能是肺母细胞瘤中具有向腺、神经内分泌分化的始基成分;胸膜肺母细胞瘤1例,是由恶性胚胎性间叶成分构成,伴有陷入的非肿瘤性上皮,此上皮表达肺表面蛋白-B.AE3、CgA、Syn、desmin、actin、CD34、TTF-1在各型肺母细胞瘤中的表达均（-）.结论肺母细胞瘤是肺原发性恶性度极高的肿瘤,诊断上应与肺的癌肉瘤、原发性肺腺癌、肺转移性子宫内膜腺癌及胚胎性横纹肌肉瘤等鉴别.
Objective To investigate the clinicopathological features, the diagnosis and differential diagnosis of pulmonary blastoma （PB）. Methods 8 cases of PB were analyzed by light microscopy, immunohistochemistry and their follow-up data. Results 8 cases aged from 5 to 63 yeats （mean 41.6）. Most masses were located in the upper lobes of left hmg or right hmg, Most patients were assessed at stage T （2-3）, N （2-3 ） and M （0）. Average survival period was about 11 months. Histologically, these tumors included 6 cases of biphasic type PB with embryonic malignant glandular showing CK positive, and the spindle stromal component positive for vimentin, nodules of cartilage positive for S-100; 1 case was apithelial type PB, It was characterized by structure ＂monda＂ with positive for NSE and AE1. The monda may be the primorditun comopnent with adenoepithelia and neuroepithelial differentiation in NB; 1 case pleuropulmonary blastoma were composed of maligant mesenchymal embryonic stroma with non-ncoplastic epithelitun expressing pulmonary surface protein B. The tumor cells were negative for CgA, Syn, desmin, actin, CD34 and TTF-1 in all cases. Conclusion The pulmonary blastoma is a high-grade primary malignant tumor with poor-prognosis, and It should be distinguished from cancer and embryonal rhabdomyosarcoma of lung. , pulmonary adenocarcinoma, metastatic endometrial
出处
《诊断病理学杂志》
CSCD
2006年第3期180-183,i0005,共5页
Chinese Journal of Diagnostic Pathology
关键词
肺母细胞瘤
免疫组化
病理诊断
鉴别诊断
Pulmonary blastoma
Immtmohistochemistry
Pathologic diagnosis
Differential diagnosis
