Raney R B, Donaldson M H, Sutow W W, Lindberg R D, Maurer H M, Tefft M
Natl Cancer Inst Monogr. 1981 Apr(56):69-74.
Rhabdomyosarcomas (RMS) arising in the head and neck region, retroperitoneum, and perineum were considered together here because the usual surgical approach is incisional biopsy. Thus successful treatment of these neoplasms depends on effective radiation therapy and chemotherapy. From November 1972 through December 1976 the Intergroup Rhabdomyosarcoma Study accrued 127 patients with primary tumors in the head and neck, 34 with orbital tumors, 24 with sarcomas arising in the retroperitoneum-pelvis (no genitourinary), and 11 with perineal lesions. Results of treatment varied among these primary sites. Patients with orbital RMS had the best prognoses; 77% of them were free of disease, compared with a 51% disease-free rate in patients with nonorbital head and neck RMS. In this latter group, disease recurrence was evenly divided among local failure, distant metastases, and direct meningeal extension. Prognoses were similar for retroperitoneal tumors; 46% of such patients are currently free of disease. That the perineum was a rare site for RMS was fortunate because only 3 of 11 such patients (27%) are free of detectable disease now (January 1979). We concluded that the site of the primary tumor is an important prognostic variable in children with RMS.
头颈部、腹膜后和会阴部位发生的横纹肌肉瘤（RMS）在此一并讨论，因为通常的手术方式是切开活检。因此，这些肿瘤的成功治疗取决于有效的放射治疗和化疗。从1972年11月到1976年12月，横纹肌肉瘤协作组研究纳入了127例原发肿瘤位于头颈部的患者、34例眼眶肿瘤患者、24例发生于腹膜后 - 骨盆（无泌尿生殖系统）的肉瘤患者以及11例会阴病变患者。这些原发部位的治疗结果各不相同。眼眶横纹肌肉瘤患者的预后最佳；其中77%无疾病，而非眼眶头颈部横纹肌肉瘤患者的无病生存率为51%。在后一组中，疾病复发在局部失败、远处转移和直接脑膜扩展之间平均分布。腹膜后肿瘤的预后相似；目前46%的此类患者无疾病。会阴是横纹肌肉瘤的罕见发病部位，这很幸运，因为在11例此类患者中，目前只有3例（27%）没有可检测到的疾病（1979年1月）。我们得出结论，原发肿瘤的部位是横纹肌肉瘤患儿重要的预后变量。
