Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: Recent experience at the Istituto Rizzoli

Gaetano Bacci, Stefano Ferrari, Franco Bertoni, Davide Donati, Patrizia Bacchini, Alessandra Longhi, Adalberto Brach del Prever, Cristiana Forni, Simonetta Rimondini

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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, ifosfamide, 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. To 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 chemotherapy-related toxicity. It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year overall 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 American Society of Clinical Oncology.

Original languageEnglish
Pages (from-to)885-892
Number of pages8
JournalJournal of Clinical Oncology
Issue number4
Publication statusPublished - Feb 2000

ASJC Scopus subject areas

  • Cancer Research
  • Oncology


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