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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 characteristics and differential diagnosis of pulmonary blastoma. Methods Eight cases of pulmonary blastoma were collected, the patient files were reviewed and histological and immunohistochemical observation and follow-up were performed. Results Eight patients with pulmonary blastoma were 5 to 63 years old with an average of 41.6 years old. The tumors mainly located in the upper lobe of the left or right lung. The clinical stage was T (2 ~ 3), N (2 ~ 3), M 0); the average survival after 11 months. Histological and immunohistochemical observations: Biphasic pulmonary blastoma in 6 cases, embryonic gonad-like structure showed AE1 / AE3 (+), naive spindle cells were vimentin (+), cartilage differentiation area S-100 ; 1 case of epithelial pulmonary blastoma, mulberry body is characterized by immunohistochemistry showed NSE and AE1 were (+), mulberry body may be in the blastoma with gland, neuroendocrine differentiation of the basic components; One case of pleuropulmonary blastoma was composed of malignant embryonic mesenchyme components with entrapped non-neoplastic epithelium expressing pulmonary surface protein-B. The expressions of AE3, CgA, Syn, desmin, actin, CD34 and TTF-1 in all types of pulmonary blastoma were all (-). Conclusions Pulmonary blastoma is a highly malignant tumor of the lung. It should be differentiated from lung carcinoid, primary lung adenocarcinoma, lung metastatic endometrial adenocarcinoma and embryonal rhabdomyosarcoma in diagnosis.