Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Abstract

Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods: We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.

Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients

“…A significant number of patients in our study (8/23, 35%) also had metastatic disease at presentation, similar to the study by Tao et al 18 of 37 patients who found metastases at presentation in 43% patients, indicating the necessity for complete staging at the time of initial diagnosis. The most common site of metastases in our study was the lung (27%), which is in agreement with the existing literature.…”

“…3 The 5-year survival in the study by El Weshi et al 3 was 46% with 35 deaths out of 57 patients. The 3-year survival in the study by Tao et al 18 was 43%. Of the 23 patients under follow-up in our study, 9 (39%) were deceased at the time of the last follow-up at a median interval of 10 months (range, 6-20 months).…”

“…Aggressive management with multimodality treatment is recommended to improve survival rates. 18,20 The high incidence of local recurrence in earlier series was owing to the lack of post-operative radiotherapy. 3 The 5-year survival in the study by El Weshi et al 3 was 46% with 35 deaths out of 57 patients.…”

“…PET/CT imaging in seven patients demonstrated distant metastases in four patients. All the metastatic lesions were 18 F-FDG-avid with mean SUVmax of 5 (range, 3-14) (Figure 2). All the 18 F-FDGavid metastases had CT correlates except for bone metastases in one patient, which remained occult on the CT.…”

Survival of Patients with Ewing's Sarcoma in Yazd-Iran

“…The patients had obviously better disease even those had been received only surgical resection. Extraskeletal Ewing's sarcoma considers as a poor prognostic factor (Tural et al, 2012;Somarouthu et al, 2014), however the outcome of the patients in our study that mainly were skeletal was worse than that Tao HT et al (2013) reported in extraskeletal Ewing's sarcoma in China. In this study median event free survival and overall survival for entire group were 15.8 months and 30.2 months respectively.…”