Li P J
Department of pathology, Beijing Children's Hospital.
Zhonghua Bing Li Xue Za Zhi. 1992 Dec;21(6):361-3.
Pulmonary blastoma is a very rare primary tumor of the lung, which mostly occur at a late age in adults. Six cases in children under four years of age are reported in this series. The light-microscopic, immunohistochemical and electron-microscopic characteristics revealed that the elements of P.B. in childhood consist mainly of mesenchymal components, including undifferentiated large cells and small round cells, which tend to differentiate toward rhabdomyosarcoma. The epithelial components comprise only a small portion of the total tumor, and most appear as the well-demarcate branching tubular structures lined with columnar or cubiodal epithelium merging with the stroma. The degree of differentiation of mesenchymal elements is closely related to the prognosis.
肺母细胞瘤是一种非常罕见的原发性肺肿瘤,多发生于成年人的老年阶段。本系列报道了4岁以下儿童的6例病例。光镜、免疫组化和电镜特征显示,儿童肺母细胞瘤的成分主要为间充质成分,包括未分化的大细胞和小圆形细胞,这些细胞倾向于向横纹肌肉瘤分化。上皮成分仅占肿瘤总体积的一小部分,大多数表现为界限清楚的分支管状结构,内衬柱状或立方上皮,与间质融合。间充质成分的分化程度与预后密切相关。