Prognostic factors and clinical outcome of patients with Ewing’s sarcoma family of tumors in adults: multicentric study of the Anatolian Society of Medical Oncology

Abstract

The aim of this study was to evaluate prognostic factors, survival rate and the efficacy of the treatment modalities used in patients with Ewing sarcoma family of tumors (ESFT). Data of patients with ESFTs followed up at different cancer centers in Turkey between 2001 and 2010 were retrospectively analyzed. The median age of 114 patients was 26 years (range 14-66). The median follow-up was 20 months (range 1-118 months). Tumor size was between 1.5 and 14 cm (median 8 cm). Eighty-six percent of patients had localized disease at presentation, and 14 % had metastatic disease. Local therapy was surgery alone in 31 % of patients, surgery combined with radiotherapy in 41 % and radiotherapy alone in 18 %. Approximately 70 % of 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 disease-free survival and overall survival were 60 and 65 %, respectively. At univariate analysis, patients with tumor size ≥ 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 cm, high lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin. ESFTs are aggressive tumors with a high incidence of local recurrence and distant metastasis. Multimodality treatment consisting of adequate surgical resection, aggressive chemotherapy (VAC alternating with IE) and radiotherapy is recommended for patients with ESFTs.

Clinical outcomes and prognostic factors of adult’s Ewing sarcoma family of tumors: single center experience

“…reported that the rate of metastasis was 34% at the time of diagnosis [ 17 ]. An additional study in adult extraskeletal ES patients showed that 31% of the patients were metastatic at the time of diagnosis [ 18 ]. Another study in adult and adolescent extraskeletal ES patients demonstrated that 43% of patients admitted to the hospital were in the metastatic stage [ 8 ].…”

Risk factors for metastasis and poor prognosis of Ewing sarcoma: a population based study

“…Previous studies have discussed prognostic factors of Ewing sarcoma. These studies have shown that advanced age, large tumor volume, axial tumor location, as well as metastatic disease at presentation are independent risk factors for poor prognosis [5][6][7][8][9][10][11][12]. Of these, metastasis at diagnosis appears to be the most common prognostic risk factor.…”

Low Grade Chondrosarcoma: what is the best treatment? A review of literature and personal opinion

“…The main goal in oncological orthopedic surgery is to obtain a wide margin for malignant tumours as wide or radical surgical margins represent one of the most important prognostic factors (27)(28)(29)(30)(31)(32)(33). This rule could be applied to every histological type including osteosarcoma, Ewing's sarcoma or CS (30)(31)(32)(33). Even though today this type of treatment is recognized for high-grade CS, several authors propose intralesional treatment with or without local adjuvant treatment for low grade (G1) CS.…”