Extraskeletal Ewings Sarcoma Family of Tumors in Adults: Prognostic Factors and Clinical Outcome

作者:Tural Deniz*; Mandel Nil Molinas; Dervisoglu Sergulen; Dincbas Fazilet Oner; Koca Sedat; Oksuz Didem Colpan; Kantarci Fatih; Turna Hande; Selcukbiricik Fatih; Hiz Murat
来源:Japanese Journal of Clinical Oncology, 2012, 42(5): 420-426.
DOI:10.1093/jjco/hys027

摘要

The aim of this study was to evaluate prognostic factors, survival rate and the efficacy of the treatment modalities used in patients with extraskeletal Ewings sarcoma. %26lt;br%26gt;Data of patients with extraskeletal Ewings sarcoma followed up at our center between 1997 and 2010 were retrospectively analyzed. %26lt;br%26gt;The median age of 27 patients was 24 years (range, 1654 years). The median follow-up was 31.8 months (range, 6144 months). Tumor size was between 1.5 and 14 cm (median: 8 cm). Eighty-five percent of patients had localized disease at presentation and 15 had metastatic disease. Local therapy was surgery alone in 16 of patients, surgery combined with radiotherapy in 42 and radiotherapy alone in 27. All patients were treated with vincristine, doxorubicin, cyclophosphamide and actinomycin-D, alternating with ifosfamide and etoposide every 3 weeks. In patients with localized disease at presentation, the 5-year event-free survival and overall survival were 59.7 and 64.5, respectively. At univariate analysis, patients with tumor size epsilon 8 cm, high serum lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy and positive surgical margin had significantly worse event-free survival. The significant predictors of worse overall survival at univariate analysis were tumor size 8 epsilon cm, high lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin. %26lt;br%26gt;Prognostic factors were similar to primary osseous Ewings sarcomas. Adequate surgical resection, aggressive chemotherapy (vincristine, doxorubicin, cyclophosphamide and actinomycin-D alternating with ifosfamide and etoposide) and radiotherapy if indicated are the recommended therapy for patients with extraskeletal Ewings sarcoma.

  • 出版日期2012-5