小儿胸膜肺母细胞瘤临床特点及病理分析

Analysis of diagnosis and treatment of pleuropulmonary blastoma in childrent

目的探讨小儿胸膜肺母细胞瘤（pleuro．pulmonaryblastoma，PPB）的临床特点、病理学特征及早期诊治方法。方法对4例反复咳嗽，x线及CT检查发现胸腔内巨大占位性病变患儿的临床特点进行分析，l例放弃治疗，3例手术切除，病理学检查及免疫组化染色确诊为PPB，结合文献进行分析。结果肿瘤组织由原始胚胎小圆细胞及梭形细胞组成，核圆形或卵圆形，部分细胞呈巢状分布，原始细胞胞质空泡状，核深染，可见明显核分裂现象。免疫组化Vimentin（＋），Desmin（＋）。结论 小儿胸膜肺母细胞瘤是罕见的肺间质恶性肿瘤，临床表现不典型，预后差，病理学检查和免疫组织化学检查有助于诊断，应及早完全手术切除，必要时辅以术后化疗。

Objetive To.explore the characteristics in clinic and pathology of pleuropulmonary blastoma in children. Methods Four cases of Pleuropulmonary blastoma （PPB） were reported with clinic data. Three cases were reported with light microscopic observation and immunohistochemical study. Results The tumor consisted of embryonal primitive small round cells and spindle ceils. Some ceils arranged in nest-like. The nu- cleus were round or oval. These cells had less cytoplasma, clear nucleolus, and mitosis was easily seen. Immu- nohistochemical study showed vimentin and desmin positive. Conclusions PPB of children is rare embryologi- cal malignant tumor with atypical symptoms and poor prognosis. It is helpful to diagnosis and treatment by rou- tine pathological examination and immunehistochemistry. The tumor must be totally removed as early as possible ,sometlmes chemotherapy is needed.