Pappo Alberto S, Devidas Meenakshi, Jenkins Jessee, Rao Bhaskar, Marcus Robert, Thomas Patrick, Gebhardt Mark, Pratt Charles, Grier Holcombe E
The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G1X8, Canada.
J Clin Oncol. 2005 Jun 20;23(18):4031-8. doi: 10.1200/JCO.2005.03.209. Epub 2005 Mar 14.
To describe the response rate and survival of children and adolescents with unresected or metastatic nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS) treated with vincristine, ifosfamide, and doxorubicin.
Between September 1996 and June 2000, 39 eligible patients received vincristine (1.5 mg/m(2) weekly for 13 doses), ifosfamide (3 g/m(2) daily for 3 days every 3 weeks for seven cycles), doxorubicin (30 mg/m(2) daily for 2 days for six cycles), and mesna (750 mg/m(2) for four doses after ifosfamide). Granulocyte colony-stimulating factor was administered daily (5 mug/kg) after each cycle of chemotherapy. Radiotherapy was administered from weeks 7 through 12.
The median patient age at diagnosis was 11.7 years; the most common primary tumor site was lower extremity (36%); and synovial sarcoma was the predominant histology. More than three fourths of all tumors were 5 cm or greater at their largest diameters. The overall objective combined partial and complete response rate was 41% (95% CI, 25.7% to 56.7%). The estimated 3-year overall survival and progression-free survival rates (+/- standard deviation) for eligible patients were 59% +/- 8.2% and 43.6% +/- 7%, respectively. Patients with clinical group III disease had significantly better 3-year and progression-free survival rates compared with patients who presented with metastatic disease.
The vincristine, ifosfamide, and doxorubicin regimen was moderately active against pediatric NRSTS. Patients with synovial sarcoma had higher response rates than other patients, and patients with unresected disease had improved outcomes. Patients with metastatic disease continue to fare poorly, and newer approaches are indicated for these patients.
描述接受长春新碱、异环磷酰胺和阿霉素治疗的未切除或转移性非横纹肌肉瘤软组织肉瘤（NRSTS）儿童及青少年的缓解率和生存率。
1996年9月至2000年6月期间，39例符合条件的患者接受了长春新碱（1.5mg/m²，每周1次，共13剂）、异环磷酰胺（3g/m²，每3周连用3天，共7个周期）、阿霉素（30mg/m²，连用2天，共6个周期）及美司钠（异环磷酰胺后用4剂，750mg/m²）治疗。化疗的每个周期后每日给予粒细胞集落刺激因子（5μg/kg）。放疗在第7至12周进行。
诊断时患者的中位年龄为11.7岁；最常见的原发肿瘤部位是下肢（36%）；滑膜肉瘤是主要的组织学类型。所有肿瘤中超过四分之三最大直径为5cm或更大。总体客观缓解率（部分缓解和完全缓解）为41%（95%可信区间，25.7%至56.7%）。符合条件患者的估计3年总生存率和无进展生存率（±标准差）分别为59%±8.2%和43.6%±7%。与出现转移性疾病的患者相比，临床III组疾病患者的3年总生存率和无进展生存率显著更高。
长春新碱、异环磷酰胺和阿霉素方案对儿童NRSTS有中度活性。滑膜肉瘤患者的缓解率高于其他患者，未切除疾病患者的预后有所改善。转移性疾病患者的预后仍然很差，这些患者需要新的治疗方法。
