Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: Recent experience at the Istituto Rizzoli
Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: Recent experience at the Istituto Rizzoli
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DOI：
10.1200/jco.2000.18.4.885
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发表时间：
2000-02-01
影响因子：
45.3
通讯作者：
Rimondini, S
中科院分区：
文献类型：
--
作者：
Bacci, G;Ferrari, S;Rimondini, S
目的：报告44例骨非转移性周围神经外胚层肿瘤（PNET）经新辅助化疗治疗的结果。患者和方法：所有患者采用六药化疗方案（长春新碱、阿霉素、放线菌素、环磷酰胺、异环磷酰胺和依托泊苷）。局部治疗包括手术20例，手术后放疗13例，仅放疗11例。结果：平均随访4.5年（2 - 7年），23例患者（52%）无事件发生，20例复发（45%），1例死于化疗相关毒性。5年无事件生存率和总生存率分别为54.2%和62.7%。为了评估神经分化在尤文氏肉瘤家族中的预后意义，我们将这些结果与138例根据相同方案治疗的典型尤文氏肉瘤（TES）合并患者的结果进行了比较。其中103例（75%）持续无事件发生，34例（24%）复发，1例死于化疗相关毒性。由此可见，采用该方案治疗的PNET患者预后明显差于典型ES患者（5年无事件生存率，54.2% vs 70.6%, P < 0.012； 5年总生存率，62.7% vs 78.3%, P < 0.002）。结论：在研究依复发风险调节的尤文氏肉瘤新辅助治疗时，还应考虑神经分化作为一个危险因素。(C) 2000年由美国临床肿瘤学会出版。
Purpose: The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported.patients and Methods: A six-drug regimen of chemotherapy (vincristine, doxorubicin, dactinomycin, cyclophosphamide, ifosfomide, and etoposide) was administered to all patients. Local treatment consisted of surgery in 20 patients, surgery followed by radiotherapy in 13, and radiotherapy only in 11.Results: At a mean follow-up of 4.5 years (range, 2 to 7 years), 23 patients (52%) remain event-free, 20 have relapsed (45%), and one has died of chemotherapy-related toxicity. The 5-year event-free survival and overall survival were 54.2% and 62.7%, respectively. Ta assess the prognostic significance of neural differentiation in the family of Ewing's sarcoma, these results have been compared with the outcomes of 138 concomitant patients with typical Ewing's sarcoma (TES) who were treated according to the same protocol. Of these, 103 (75%) remained continuously event-free, 34 (24%) relapsed, and one died of chemothempy-related toxicity. It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year event-free survival, 54.2% v 70.6%, P < .012; 5-year overall survival, 62.7% v 78.3%, P < .002).Conclusion: The authors conclude that studies into new adjuvant therapy for Ewing's sarcoma modulated according to risk of relapse should also consider neural differentiation as a risk factor. (C) 2000 by American Society of Clinical Oncology.