Is primary thoracic ewing sarcoma aggressive than others? seventeenyears' experience

Abstract

Background: Ewing sarcoma (ES) is an uncommon aggressive malignant tumor of the bone and/or soft tissue and belongs to peripheral primitive neuroectodermal tumor (PNET) family of tumors. Bone PNETs primarily arise from the diaphysis of long bones, where as soft tissue PNETs are most commonly found in the chest wall. Multidisciplinary treatments, including chemotherapy, surgery, radiotherapy, or all three combined, improve the survival of patients with localized ES. However, the best approach to achieve local control remains controversial. Materials and Methods: We retrospectively analysed the medical records and pathology data of 14 patients (8 male, 6 female; mean age, 23.2 [range, 4-54] years) with primary thoracic ES who underwent surgery in our clinic between January 2002 and December 2019. In addition, the treatment modalities and tumor-related factors of chest wall ES and lung parenchyma were evaluated. Results: The most frequent complaint was chest pain (n = 7). In 10 patients, the tumor originated from the ribs, whereas the remaining 4 patients had lung parenchymal tumors. Ten patients underwent complete tumor excision with chest wall resection, one patient underwent lower lobectomy with chest wall resection, and three patients underwent complete tumor excision via wedge resection. All patients were treated with chemotherapy, except two who underwent bone marrow transplantation. The median follow-up was 31.6 (range, 2-84) months. Relapses were seen in 5 cases in the median 19.8th (range, 4-60) month. Conclusions: Complete tumor resection is the most effective treatment for thoracic ES and multimodal therapy (surgical resection, chemotherapy, and local radiation therapy), which is recommended when indicated, constitutes the optimal treatment for ES. Although relapses occur within the early postoperative period, late relapses are not uncommon. The follow-up periods must be short and should be maintained long term for late relapses.