Abstract
BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma. The aim of this study is to present the results of the patients we treated with the diagnosis of EMC as an oncology reference center. METHODS: Information on 13 patients diagnosed with EMC between 2006 and 2018 was retrospectively reviewed. Patients' demographic information, tumor sizes, surgical treatments, chemotherapy and radiotherapy statuses, follow-up times, recurrences, and metastases were recorded. RESULTS: Mean patient age was 53.6 ± 15 years (range: 28-73). In 8 patients, the tumor was located in the lower limbs, most commonly in the thigh (46.2%). Mean follow-up period was 52.8 ± 19.9 (24-96) months. All patients underwent wide resections and only one had a positive surgical margin. In follow-up, 5 (38.5%) patients experienced recurrence; 6 patients had lung metastasis (46.2%) and 7 patients (53.8%) died. Mean tumor size was 10.4 ± 3.2 (5-17) cm. Median survival time was 61 (50.5-71.4) months and 5-year survival rate was 51.8%. There was no significant difference between survival times according to age, gender, side, limb location, postoperative radiotherapy, recurrence, or presence of lung metastasis. The cut-off value for death obtained by ROC analysis of tumor size was 11 cm. DISCUSSION: EMC is a rare soft tissue sarcoma with high local recurrence and metastasis capacity. Tumor size and metastatic disease are poor prognostic criteria. If it is a localized disease, the first option should be wide resection.