Journal
PEDIATRIC BLOOD & CANCER
Volume 49, Issue 7, Pages 894-900Publisher
WILEY-LISS
DOI: 10.1002/pbc.21233
Keywords
alkylating agents; bone tumors; dose intensity; metastatic ewing sarcoma; metastatic PNET; oxazaphosphorine chemotherapy
Categories
Funding
- NCI NIH HHS [U10 CA 98543, U10 CA 13539, U10 CA 30969] Funding Source: Medline
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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.
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