Subject(s)
Erdheim-Chester Disease , Urology , Humans , Erdheim-Chester Disease/diagnosis , Diagnosis, DifferentialABSTRACT
Extraosseous Ewing sarcoma is a rare and aggressive malignancy belonging to the Ewing sarcoma family of tumors, primarily affecting soft tissues such as the pelvis, retroperitoneum, and chest wall. Although it predominantly involves these soft tissues, extraosseous Ewing sarcoma can also occur in solid organs, including the pancreas. Here, we present a rare case of a 4-year-old girl diagnosed with primary extraosseous Ewing sarcoma of the pancreas.
ABSTRACT
INTRODUCTION: This study aimed to analyse the effect of preoperative radiotherapy on survival, recurrence and necrosis rates in osteosarcoma patients treated with neoadjuvant chemotherapy and limb-salvage surgery. MATERIALS AND METHODS: Osteosarcoma patients who referred to our clinic from 1998 to 2015 were investigated retrospectively. 141 patients with non-metastatic osteosarcoma of the limb who received neoadjuvant chemotherapy and underwent limb-sparing surgery with or without preoperative radiotherapy were included in the study. 73 patients had neoadjuvant chemotherapy only (CT group), while 68 patients had additional preoperative radiotherapy (CT + RT group). 5-year survival, disease-free survival, limb survival, local recurrence, metastasis, complication and necrosis rates were compared between the two groups. RESULTS: Overall 5-year survival, 5-year disease-free survival, 5-year limb survival rates were 68.5, 64.3, 65.7 for CT group and 72.1, 67.6, 64.7 for CT + RT group, respectively. Preoperative radiotherapy gave similar survival and disease-free survival rates. Limb survival, recurrence and metastasis rates were similar between the groups. The necrosis rate of the lesions was significantly higher in the CT + RT group compared to the CT group. CONCLUSION: Preoperative radiotherapy can provide a higher necrosis rate and may allow the resection of unresectable lesions in the treatment of osteosarcoma. CT + RT provided similar 5-year survival despite the larger tumour size compared to CT group. Due to the higher wound complication rate (8.2% in CT, 23.5% in CT + RT group, p = 0.01), CT + RT should be restricted to the lesions with large size or close proximity to neurovascular structures. LEVEL OF EVIDENCE: Level III; Therapeutic, Retrospective comparative study.
