联合CDV与IE化疗方案治疗儿童外周原始神经外胚层肿瘤

目的：观察联合CDV（环磷酰胺＋吡柔比星＋长春新碱）与IE（异环磷酰胺＋依托泊苷）化疗方案治疗儿童外周原始神经外胚层肿瘤（pPNET）的疗效及安全性。方法：回顾分析CDV与IE方案治疗12例pPNET患儿的临床资料,采用WHO实体瘤客观疗效评定标准和美国国立癌症研究所通用不良反应分级标准对化疗疗效和相关毒副反应进行评价。结果：12例中完全缓解7例（58.3%）、部分缓解3例（25.0%）、稳定0例、进展1例（8.3%）、复发5例（41.7%）。患儿骨髓均有Ⅲ、Ⅳ级毒性损害;1例死于感染;消化道、肝脏、肾脏损害为Ⅰ、Ⅱ级;1例Ⅰ级神经毒性病例;无心功能受损病例。结论：CDV与IE联合化疗方案可以有效、安全的治疗儿童外周原始神经外胚层肿瘤。

Treatment of childhood peripheral primitive neuroectodermal tumors by CDV and IE chemotherapy

Objective：To evaluate the therapeutic effects and chemotherapy-related toxicity of CDV（Cyclophosphamide＋Doxorubicin＋Vincristine） and IE（ifosamide＋Etoposide） protocol in the treatment of childhood peripheral primitive neuroectodermal tumors（pPNET）.Methods：The clinical data of 12 patients aged from 1.5 to 12 years old with pPNET was analyzed retrospectively.All patients were treated with CDV and IE chemotherapy.The therapeutic response evaluation criterion of WHO and the classification criteria for adverse events of National Cancer Institute was used to assess the therapeutic response and chemotherapy-related toxicity.Results：Among 12 patients underwent CDV and IE chemotherapy,there were 7 complete response（58.3%）,3 partial response（25.0%）,and 5 relapses（41.7%）.One patient developed severer（8.3%）.All patients had bone marrow suppression at grade III or IV,and suffered lesions at digestive tract,liver or kidney at grade I or II.One patient developed gradeⅠneurotoxicity and one died of infection in lung.No lesion was found in heart function.Conclusion：The combination of CDV and IE chemotherapy is effective and safe in treatment of childhood pPNET.