Schiavetti A, Dominici C, Matrunola M, Capocaccia P, Ceccamea A, Castello M A
Department of Pediatrics, University La Sapienza, Rome, Italy.
Med Pediatr Oncol. 1996 Mar;26(3):201-7. doi: 10.1002/(SICI)1096-911X(199603)26:3<201::AID-MPO10>3.0.CO;2-J.
The cases of two children under three years of age with primary pulmonary rhabdomyosarcoma and no associated lung malformations are reported and a review of the literature is presented. In both, complete surgical removal of the tumor was performed and histologic examination revealed embryonal subtype. Flow cytometric assessment showed a tumor-cell diploid DNA content. Postoperative radio- and chemotherapy were carried out, but in spite of treatment both girls died because of disease progression, fourteen and nine months after diagnosis. The importance of associated cystic lung malformations and DNA content in predicting clinical outcome of primary pulmonary rhabdomyosarcoma is evaluated.
报告了两例三岁以下原发性肺横纹肌肉瘤且无相关肺部畸形的儿童病例，并对文献进行了综述。两例均进行了肿瘤的完整手术切除，组织学检查显示为胚胎型亚型。流式细胞术评估显示肿瘤细胞DNA含量为二倍体。术后进行了放疗和化疗，但尽管进行了治疗，两个女孩均因疾病进展在诊断后14个月和9个月死亡。评估了相关肺囊性畸形和DNA含量在预测原发性肺横纹肌肉瘤临床结局中的重要性。
