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Background: The choice of a local control (LC) modality for Ewing sarcoma (EWS) of the femur is controversial. This study aimed to determine the effect of LC modality on tumor LC and patient outcomes., Methods: The study reviewed the treatment and outcomes for 115 patients who had EWS of the femur treated with similar chemotherapy in three cooperative group trials. Patient outcomes were analyzed according to the LC modality using the log-rank test and the cumulative incidence of local or distant failure using competing risks regression., Results: The median age of the patients was 13 years. The most common tumor location was the proximal femur followed by the mid femur. For 55 patients with available data, the tumor was larger than 8 cm in 29 patients and 8 cm or smaller in 26 patients. For 84 patients (73 %), surgery only was performed, whereas 17 patients (15 %) had surgery plus radiation, and 14 patients (12 %) had radiation only. The 5-year event-free survival (EFS) rate was 65 % (95 % confidence interval [CI], 55-73 %), and the 5-year overall survival (OS) rate was 70 % (95 % CI, 61-78 %). Patient outcomes did not differ significantly according to tumor location within the femur (proximal, mid or distal) or tumor size (<8 vs ≥8 cm). The findings showed no statistically significant differences in EFS, OS, cumulative incidence of local failure, or cumulative incidence of distant failure according to LC modality (surgery, surgery plus radiation, or radiation)., Conclusions: The LC modality did not significantly affect disease outcome for EWS of the femur. Further study of treatment complications and functional outcome may help to define the optimal LC modality., Competing Interests: There are no conflicts of interest.

Background: Adults with metastatic Ewing family sarcomas (ES) after being treated with standard therapy for localised disease have limited treatment options with curative intent. The aim of this retrospective single centre study was to evaluate the efficacy of autologous stem cell transplantation (ASCT) in this patient population., Methods: We report on seven consecutive patients with ES. Four patients with initial localised disease developed distant metastases after being treated with initial standard pre-operative chemotherapy followed by surgery and subsequent standard post-operative chemotherapy. Three patients with initial metastatic disease were pre-treated with standard polychemotherapy. All patients received high-dose chemotherapy (HDCT) followed by ASCT for metastatic disease., Results: For patients with initial metastatic disease, partial remission (PR) was achieved in three patients prior to HDCT. Type of response subsequent to ASCT was complete response (CR) in four patients with initial localised disease and CR in two patients with initial metastatic disease. No patient died within the first 100 days after HDCT. Side effects were rare and manageable., Conclusion: This retrospective analysis suggests that ASCT may be considered in patients with metastatic ES, previously treated with standard therapy.

Background: Advances in the treatment of Ewing sarcoma family of tumors (ESFT) are the result of improvements in systemic and local therapies. Clinical data of extraosseous ESFT are scarce., Methods: A retrospective analysis of all patients with extraosseous ESFT treated at St. Jude Children's Research Hospital (SJCRH) from June 1982 to August 2009., Results: Forty-six patients with extraosseous ESFT were identified. The mean age at diagnosis was 13.8 years. The majority of patients were male and white. The most common site of primary tumor was the trunk. Twelve patients had subcutaneous tumors. The median tumor size was 8 cm. Six patients (13 %) had metastatic disease at diagnosis. A total of 59 % of patients were alive at the time of analysis, with a median follow-up from diagnosis of 15.3 years. Fifteen-year estimates of survival and event-free survival (EFS) for all patients were 53.3 ± 9.4 and 50 ± 9.1 %, respectively. Fifteen-year estimates of survival and EFS with localized disease were 61.4 ± 9.8 and 57.6 ± 9.7 %, respectively. Stage and subcutaneous ESFT were significant predictors of outcome. There was no significant difference in patient's demographics and tumor characteristics between patients with skeletal ESFT and extraosseous Ewing sarcoma. The outcome for patients with localized extraosseous Ewing sarcoma was similar to that reported for all localized ESFT patients treated at SJCRH., Conclusions: The outcome for localized patients treated with extraosseous ESFT was similar to that reported for all ESFT patients treated on protocols at SJCRH. Patients with subcutaneous ESFT had a favorable prognosis when compared to their counterparts.

The overall prognosis of relapsing Ewing sarcoma is poor and therapeutic options can be limited by extensive chemotherapeutic pretreatment. We report on a case of a 27-year-old male, presenting with a large mediastinal mass and malignant pleural effusions. 5 years prior peripheral Ewing sarcoma had been treated according to the CESS 86 protocol. Relapse chemotherapy was initiated (CESS protocol) but tumor progression led to stenoses of both main bronchi. At this critical point, 2 Strecker tantal stents were placed endoscopically to prevent suffocation and provide the time for further chemotherapy, regardless of the poor overall prognosis. Complete remission was achieved by high-dose ifosfamide, surgery, radiotherapy and adjuvant ifosfamide. In spite of possible complications of long-term stent implantation, the stents were not removed until 4 years later when stent dislocation occurred. After removal, the stents were epithelialized and electron microscopy demonstrated structural integrity of the stent. The patient has remained in complete remission since (6 years).

Ewing's sarcoma is one of the malignant bone tumours, which occurs in various bones of the body. When the spinal column or skull bones are involved, it may cause spinal cord compression or brain invasion. In this study, 12 cases of Ewing's sarcoma with central nervous system (CNS) involvement are presented. Seven of them were male and five female. In seven of the cases tumoural mass was located in the spinal column, whereas in the others it was found in the cranium. The major complaints were motor weakness in the lower extremities in seven cases, and a mass on the cranial vault in four cases. On neurological examination, eight of the patients had impairment of movements of the extremities (67%), and seven had sensory loss (58%). All except one patient underwent surgery. In seven cases, decompressive laminectomy plus subtotal tumour excision was performed, in three, craniectomy with grossly total tumour excision, and in one, biopsy. In this report, age and sex distribution, complaints, clinical and laboratory findings, neurological examination, and histopathology of our cases are reviewed and surgical results are compared with others reported in the literature.

The evidence of CNS involvement in Ewing's sarcoma is uncommon and very often due to a direct extension of the tumor to extradural space and meninges. We report a case of a 14-year old girl with a primary ischio-pubic localization of Ewing's sarcoma. Body CT scan showed a bone lesion involving the left fronto-temporal region of the skull without affecting the brain. After chemotherapy radiological findings showed complete recovery. Ten months after treatment, the patient had neurological symptoms. Cerebral CT scan evidenced a large tumor mass localized in the right temporal lobe. Following radiotherapy the tumor mass was no longer visible. This case shows that late intraparenchymal CNS metastasis occurs independently from the previous skull lesion. Therefore these data, even through based on a single observation, lead to believe that in some cases prophylactic chemotherapy and radiotherapy of CNS may not be necessary.