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儿童原发性肺横纹肌肉瘤。两例以自发性气胸表现的病例报告。

Primary pulmonary rhabdomyosarcoma of the lung in children. Report of two cases presenting with spontaneous pneumothorax.

作者信息

Allan B T, Day D L, Dehner L P

出版信息

Cancer. 1987 Mar 1;59(5):1005-11. doi: 10.1002/1097-0142(19870301)59:5<1005::aid-cncr2820590526>3.0.co;2-5.

DOI:10.1002/1097-0142(19870301)59:5<1005::aid-cncr2820590526>3.0.co;2-5
PMID:3815259
Abstract

This report describes two cases of pulmonary rhabdomyosarcoma occurring in children, ages 21 and 30 months at diagnosis. Both patients presented with spontaneous pneumothorax and had cystic changes in the affected lung by chest x-ray. Ultrastructural and immunohistochemical studies supported the myogenic phenotype of these neoplasms. The authors were unable to confirm that either of these tumors had unequivocally originated in a congenital cyst although previous authors have reported this association. An apparent morphologic spectrum of embryonic appearing neoplasms including pulmonary blastoma and a malignant mesenchymoma have been observed in the lungs of children.

摘要

本报告描述了两例儿童肺横纹肌肉瘤病例,诊断时年龄分别为21个月和30个月。两名患者均表现为自发性气胸,胸部X线检查显示患侧肺有囊性改变。超微结构和免疫组织化学研究支持这些肿瘤的肌源性表型。尽管此前有作者报道过这种关联,但作者无法证实这两种肿瘤中有任何一种明确起源于先天性囊肿。在儿童肺部观察到明显的形态学谱,包括肺母细胞瘤和恶性间叶瘤等胚胎样肿瘤。

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