Surgical outcomes of extraskeletal myxoid chondrosarcoma

BACKGROUND/AIM: 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. MATERIALS AND 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. CONCLUSION: 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.