摘要
目的 :阐明胸膜肺母细胞瘤的病理形态特征及鉴别诊断要点。方法 :对 1例小儿胸膜肺母细胞瘤进行了组织形态学、免疫组织化学分析。结果 :胸膜肺母细胞瘤是一种纯间质性恶性肿瘤 ,好发于 12岁以下的儿童及婴幼儿 ,起源于肺、胸膜或纵隔的原始细胞 ,镜下可见原始胚胎性的圆形或短梭形细胞 ,瘤细胞有呈巢趋势 ,酷似肾母细胞瘤、肝母细胞瘤等肿瘤原始胚基成分 ,免疫组化未见有上皮细胞及其他间叶成分标志的表达。另一种成分为梭形细胞 ,除表现为纤维肉瘤特点外 ,还向横纹肌肉瘤分化。结论 :胸膜肺母细胞瘤是一种极为罕见的恶性肿瘤 ,应与肺母细胞瘤、原始神经外胚叶肿瘤及胚胎性横纹肌肉瘤等鉴别。
Purpose To investigate the pathomorphologic features and differential diagnosis of pleuropulmonary blastoma(PPB). Methods A case of PPB in a boy aged 7 was reported with light microscopic observation and an immunohistochemical study. Results PPB was a malignant mesenchynal tumor and occurred most commonly in children under 12 years old. The tumor arose from primitive cells of lung, pleura and mediastinum. The tumor consisted of primitive small cells, negative both for epithelial and special mesenchymal differentiated markers by immunohistochemistry, and variety of differentiated and /or anaplastic spindle sarcomatous elements including fibrosarcoma and embryonal rhabdomyosarcoma. Conclusions PPB is a rare malignant tumor and should be distinguished from pulmonary blastoma, primitive neuroectoural tumor (PNET), embryonal rhabdomyosarcoma and other sarcomas.
出处
《临床与实验病理学杂志》
CAS
CSCD
2001年第6期486-489,共4页
Chinese Journal of Clinical and Experimental Pathology
