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Extraskeletal myxoid chondrosarcoma: Clinicopathological features and outcomes from the United States sarcoma collaborative database. J Surg Oncol 2022 Dec;126(8):1533-1542



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BACKGROUNDS AND OBJECTIVES: This investigation described clinicopathological features and outcomes of extraskeletal myxoid chondrosarcoma (EMC) patients.

METHODS: EMC patients were identified from the United States Sarcoma Collaborative database between 2000 and 2016. Overall survival (OS) and recurrence-free survival (RFS) were calculated, and prognostic factors were analyzed.

RESULTS: Sixty individuals with a mean age of 55 years were included, and 65.0% (n = 39) were male. 73.3% (n = 44) had a primary tumor. A total of 41.6% (n = 25) developed tumor relapse following resection. The locoregional recurrence rate was 30.0% (n = 18/60), and mean follow-up was 42.7 months. The 5-year OS was 71.0%, while the 5-year RFS was 41.4%. On multivariate analysis for all EMC, chemotherapy (hazard ratio [HR], 6.054; 95% confidence interval [CI], 1.33-27.7; p = 0.020) and radiation (HR, 5.07, 95% CI, 1.3-20.1; p = 0.021) were independently predictive of a worse RFS. Among patients with primary EMC only, the 5-year OS was 85.3%, with a 30.0% (n = 12) locoregional recurrence rate, though no significant prognostic factors were identified.

CONCLUSIONS: Long-term survival with EMC is probable, however there exists a high incidence of locoregional recurrence. While chemotherapy and radiation were associated with a worse RFS, these findings were likely confounded by recurrent disease as significance was lost in the primary EMC-only subset.

Author List

Gusho CA, King D, Ethun CG, Cardona K, Harrison Howard J, Tran TB, Poultsides G, Tseng J, Roggin KK, Fields RC, Cullinan DR, Chouliaras K, Votanopoulos K, Grignol VP, Bedi M


Manpreet Bedi MD, MS Associate Professor in the Radiation Oncology department at Medical College of Wisconsin
David M. King MD Chair, Professor in the Orthopaedic Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Middle Aged
Neoplasms, Connective and Soft Tissue
Soft Tissue Neoplasms
United States