胸膜肺母细胞瘤基础研究与诊治进展

Advance of Basic Research and Clinical Diagnosis and Treatment of Pleuropulmonary Blastoma

胸膜肺母细胞瘤(PPB)是好发于儿童的一种罕见恶性肿瘤,在病史、基因遗传学特征、临床表现及预后方面有一定特点。PPB常累及胸膜和肺,具有特殊的病理生理学特征和复杂的生物学行为,常发生于5岁以下儿童。其病理学特点为肿瘤间质内有大量原始小圆细胞及纺锤状细胞,肿瘤组织的上皮细胞为良性呼吸道上皮细胞。该文就近年来PPB的病理生理学特征、分子遗传学特征、临床表现、临床诊断与鉴别诊断、治疗及预后等研究进展进行综述。

Pleuropuimonary blastoma（PPB） is a kind of rare malignancy which often occursin children. It is quite special in histology, genetic characteristics, clinical manifestations and prognosis. It often involves pleura and lung and has special pathophysiological characteristics and complex biological behaviors. It mostly affects children under the age of five. The pathological feature is containing a large number of embryoual primitive small round cells and spindle cells in mesenchyme and the epithelial cells of the tumor tissue are benign airway epithelial cells. Here is to make a review of the research progress of the pathophysiological char- acteristics, molecular genetic characteristics, clinical manifestations, clinical diagnosis and differential diagno- sis, and treatment and prognosis