Histological and overall response to ICE-CAV dose intensification in newly diagnosed high-risk Ewing sarcoma family tumors: the Bambino Gesù Childrens Hospital experience

Abstract BACKGROUND: Ewing sarcoma (ES) and extraosseous ES/primitive neuroectodermal tumors (PNET) share histopathologic features of the ES family of tumors (ESFT). The authors report on their results from a regimen of ifosfamide, carboplatin, and etoposide (ICE) with cyclophosphamide, doxorubicin, and vincristine (CAV) dose intensification in patients with high-risk ESFT. METHODS: Since 1990, patients with ESFT and with 1 or more of the following risk factors were reviewed: tumor volume > 200 mL, tumor site with a poor prognosis, and pulmonary and/or bone marrow metastases. RESULTS: Thirty-six patients with ESFT who were involved in the study were divided into 2 arms of 18 patients each. One group received treatment with various regimens, and the other group received treatment with ICE plus CAV. The disease was brought under control more rapidly in the latter patients, for whom surgery was more easily feasible, and up to 90% of patients achieved a major response, with an estimated 3-year overall survival rate of 67% +/- 12%. CONCLUSIONS: The current results showed that ICE plus CAV was tolerated well and was effective in the studied subset of tumors, indicating that dose intensification correlates with better disease control, a high percentage of necrosis, and conservative surgery in patients with high-risk ESFT.