肺母细胞瘤与胸膜肺母细胞瘤的诊断及鉴别诊断

Diagnosis and differential diagnosis of pulmonary blastoma and pleuropulmonary blastoma

目的分析并鉴别肺母细胞瘤与胸膜肺母细胞瘤的临床特征。方法收集2010年1月至2017年10月郑州大学第一附属医院肺母细胞瘤与胸膜肺母细胞瘤患者的临床资料,共4例(肺母细胞瘤3例,胸膜肺母细胞瘤1例),回顾性分析其临床及病理学资料,并复习相关文献总结各自的临床病理特征并加以鉴别。结果临床特征:4例患者有不同程度的咳嗽、咯血、下肢水肿、四肢乏力等症状,红细胞、血红蛋白均降低,纤维蛋白原均升高。病理特征:肺母细胞瘤由高分化腺癌及原始间胚叶构成,腺癌由复层柱状上皮构成,可见核上及核下空泡,原始间胚叶成分可见横纹肌分化及黏液样变;胸膜肺母细胞瘤可见囊壁样结构,囊壁被覆分化成熟的纤毛柱状上皮,间叶成分由丰富的圆形和梭形幼稚成分构成,可见软骨化生。免疫组化:3例肺母细胞瘤CK、β-Catenin、vimentin表达均阳性,Ki67阳性率均在50%以上; 1例胸膜肺母细胞瘤上皮成分CK(+),有软骨分化区域S-100(+),Ki67(90%+)。结论肺母细胞瘤是常见于成人的由腺癌及间叶组织构成的具有双向分化的肿瘤,上皮及间叶均为恶性成分。胸膜肺母细胞瘤是一种好发于儿童的由恶性间质构成的单相型肿瘤,其间质异形性显著,恶性程度较高。免疫组化无特异性表达可鉴别二者,病理诊断主要依据形态学加以鉴别。

Objective To analyze and identify of clinical features of pulmonary blastoma and pleuropulmonary blastoma. Methods The clinical data of 4 patients with pulmonary blastoma or pleuropulmonary blastoma in the First Affiliated Hospital of Zhengzhou University from January of 2010 to October of 2017 were collected, including 3 cases of pulmonary blastoma and 1 case of pleuropulmonary blastoma. The clinical and pathological data were analyzed retrospectively, and the relevant literatures were reviewed to summarize their clinical and pathological characteristics and identify them. Results In terms of clinical features,4 patients had cough, hemoptysis, lower extremity edema, limb weakness and other symptoms in varying degrees.Erythrocyte and hemoglobin decreased and fibrinogen increased. In terms of pathological features, the pulmonary blastoma was composed of two components of highly differentiated adenocarcinoma and primitive mesoderm. Adenocarcinoma was composed of complex layer columnar epithelium with vacuoles above and below the nucleus. Rhabdomyosus differentiation and mucoid change could be seen in the original mesoderm components. Cyst-like structure could be seen in the pleuropulmonary blastoma, and the wall was covered with mature ciliated columnar epithelium. The components of the mesenchymal stem were composed of rich round and spindle naive components, and cartilaginous metaplasia was visible . Three cases of pulmonary blastoma showed positive expression of CK,β-Catenin and vimentin, and the positive rate of Ki67 was above 50%. One case of pleuropulmonary blastoma had epithelial component CK (+), cartilage differentiation area S-100 (+) and Ki67 (90%+). Conclusion Pulmonary blastoma is a biphasic tumor composed of adenocarcinoma and mesenchymal tissue, which is seen commonly in adults. Both epithelial and mesenchymal are malignant components. Pleuropulmonary blastoma is seen frequently in children,which is a kind of single phase tumor composed of malignant stroma. The interstitial heteromorphism of the pleuropulmonary blastoma is significant and highly malignant. Immunohistochemical staining is not specific to identify each of them, and pathological diagnosis is mainly based on morphology.