Treatment of metastatuc Ewing Sarcoma/Primitive neuroectodermal tumor of bone: Evaluation of increasing the dose intensity of chemotherapy - A report from the children's oncology group
Treatment of metastatuc Ewing Sarcoma/Primitive neuroectodermal tumor of bone: Evaluation of increasing the dose intensity of chemotherapy - A report from the children's oncology group
复制标题
DOI：
10.1002/pbc.21233
复制
发表时间：
2007-12-01
影响因子：
3.2
通讯作者：
Grier, Holcombe E.
中科院分区：
文献类型：
--
作者：
Miser, James S.;Goldsby, Robert E.;Grier, Holcombe E.
背景。尽管采用了新的治疗方法，但诊断时患有骨转移的尤文肉瘤家族肿瘤 (ESFT) 患者的结果仍然很差。我们评估了剂量强度化疗方案是否可以提高诊断时患有骨转移的 ESFT 患者的生存率。方法。我们对 60 名患有骨转移性 ESFT 的患者进行了一项新强化治疗的单臂试验。治疗包括 51 周的化疗和通过放疗、手术或两者结合对原发灶进行局部控制。化疗方案包括两种交替治疗方案：一种是长春新碱 (2 mg/m(2))、阿霉素 (90 mg/m2) 和环磷酰胺 (2,200 mg/m(2))；另一种是长春新碱 (2 mg/m(2))、阿霉素 (90 mg/m2) 和环磷酰胺 (2,200 mg/m(2))。第二个是异环磷酰胺（2,800 mg/m(2)/天 x 5 天）和依托泊苷（100 mg/m(2)/天 x 5 天）。结果。在参加这项单臂试验的 60 名骨转移性 ESFT 患者中，12 名仅转移至肺，7 名仅转移至骨髓或骨，38 名转移至多个部位，2 名转移至其他部位，3 名未指定。共有三人中毒死亡。 6 名患者（6 年累积发病率：9%）出现第二恶性肿瘤并死亡。 6 年总体无事件生存率 (EFS) 为 28%（标准误差 (SE) 6%），生存率 (S) 为 29% (SE 6%)。结论。使用更高剂量的环磷酰胺、异环磷酰胺和阿霉素的强化治疗方案增加了毒性和第二恶性肿瘤的风险，但没有改善 EFS 和 S。
Background. The outcome for patients with Ewing sarcoma family of tumors (ESFTs) of bone with metastases at diagnosis remains poor despite new approaches to treatment. We evaluated whether a dose-intensity chemotherapy regimen improved survival for patients with ESFTs of bone with metastases at diagnosis. Methods. We entered 60 patients with metastatic ESFTs of bone onto a single arm trial of a new intensive therapy. Treatment consisted of 51-weeks of chemotherapy and local control of the primary with radiation, surgery, or both. The chemotherapeutic protocol included two alternating blocks: one with vincristine (2 mg/m(2)), doxorubicin (90 mg/m2), and cyclophosphamide (2,200 mg/m(2)); and the second with ifosfamide (2,800 mg/m(2)/day x 5 days) and etoposide (100 mg/m(2)/day x 5 days). Results. Of the 60 patients with metastatic ESFTs of bone enrolled onto this single arm trial, 12 had metastasis to lung only, 7 to bone marrow or bone only, 38 to multiple sites, 2 in other sites and 3 not specified. There were three toxic deaths. Six patients (6-year cumulative incidence: 9%) developed second malignant neoplasms and died. The 6-year overall event-free survival (EFS) was 28% (standard error (SE) 6%) and survival (S) was 29% (SE 6%). Conclusion. An intensified treatment regimen using higher doses of cyclophosphainide, ifosfamide, and doxorubicin increased toxicity and risk of second malignancy without improving EFS and S.