Raney R B, Crist W, Hays D, Newton W, Ruymann F, Tefft M, Beltangady M
IRS Committee of the Pediatric Oncology Group, St. Louis, MO.
Cancer. 1990 Jun 15;65(12):2787-92. doi: 10.1002/1097-0142(19900615)65:12<2787::aid-cncr2820651230>3.0.co;2-k.
Thirty-six previously untreated patients younger than 21 years of age with sarcoma arising in the perineal region were entered on the Intergroup Rhabdomyosarcoma Studies (IRS) I and II from 1972 through 1984. The tumor histologic subtype was alveolar rhabdomyosarcoma (RMS) in 20 patients (56%), embryonal RMS in 11 patients (30%), and other types of sarcoma in 5 patients (14%). Fifteen children had grossly complete surgical excision (Clinical Groups I and II), and 15 had localized gross residual tumor (Group III) after initial operative management. Six patients had distant metastases (Group IV) at diagnosis. Twelve patients without distant metastases underwent regional lymph node biopsy; tumor involvement was found in six. Subsequent treatment consisted of chemotherapy with vincristine (VCR) and dactinomycin (AMD) for all patients; patients in Groups III and IV also received cyclophosphamide (CYC) with or without Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH). Radiation therapy (RT) was administered to patients in Clinical Groups II, III, and IV. Overall, 28 (78%) patients achieved a complete clinical response. The 3-year disease-free survival rate for these patients was 42%, compared with 52% for all other patients in the combined IRS I and IRS II series (P = 0.44). The overall 3-year survival rate was 59%, compared with 64% for all other patients in IRS I and IRS II (P = 0.48). Aggressive treatment is needed in children with perineal sarcoma to improve their prognosis.
1972年至1984年期间，36例年龄小于21岁、会阴区原发未接受过治疗的肉瘤患者被纳入横纹肌肉瘤协作组研究（IRS）I和II。肿瘤组织学亚型为肺泡状横纹肌肉瘤（RMS）20例（56%），胚胎型RMS 11例（30%），其他类型肉瘤5例（14%）。15例患儿接受了大体完整的手术切除（临床I组和II组），15例在初始手术治疗后有局限性大体残留肿瘤（III组）。6例患者在诊断时已有远处转移（IV组）。12例无远处转移的患者接受了区域淋巴结活检，其中6例发现有肿瘤累及。所有患者随后均接受了长春新碱（VCR）和放线菌素D（AMD）化疗；III组和IV组患者还接受了环磷酰胺（CYC），加或不加阿霉素（ADR）（多柔比星；俄亥俄州哥伦布市的Adria实验室）。临床II组、III组和IV组患者接受了放射治疗（RT）。总体而言，28例（78%）患者获得了完全临床缓解。这些患者的3年无病生存率为42%，而IRS I和IRS II联合系列中的所有其他患者为52%（P = 0.44）。总体3年生存率为59%，而IRS I和IRS II中的所有其他患者为64%（P = 0.48）。会阴肉瘤患儿需要积极治疗以改善预后。
