Extraskeletal myxoid chondrosarcoma: A review of 23 patients treated at a single referral center with long-term follow-up

Background :Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma.Although it has been regarded as a low-grade sarcoma unassociated with tumorrelated death, a recent study has suggested an insidious nature with a high propensity for relapse during a long disease course. The aim of this study was to clarify the long-term clinical features of EMC treated at a single referral center using state-of-the-art techniques. Methods A retrospective review of 23 consecutive patients (10 males, 13 females; mean age 58 years) treated between 1979 and 2008 (mean follow-up; 109 months) was performed. Results :Surgery for the primary tumor was performed in 22 patients, and 7 cases recurred locally due to inadequate resection. Eleven patients had metastatic disease, either at diagnosis (3) or developing later (8). The 5/10-year overall survival rates were 91/84 %, and the 5/10-year local recurrence-free and metastasis-free survival rates for patients with localized disease were 89/62 and 89/61 %, respectively. Larger tumor size ([10 cm) and metastases at diagnosis were significant negative prognostic factors. Four.