TY - JOUR
T1 - Extraskeletal myxoid chondrosarcoma
T2 - Clinicopathological features and outcomes from the United States sarcoma collaborative database
AU - Gusho, Charles A.
AU - King, David
AU - Ethun, Cecilia G.
AU - Cardona, Kenneth
AU - Harrison Howard, J.
AU - Tran, Thuy B.
AU - Poultsides, George
AU - Tseng, Jennifer
AU - Roggin, Kevin K.
AU - Fields, Ryan C.
AU - Cullinan, Darren R.
AU - Chouliaras, Konstantinos
AU - Votanopoulos, Konstantinos
AU - Grignol, Valerie P.
AU - Bedi, Meena
N1 - Publisher Copyright:
© 2022 Wiley Periodicals LLC.
PY - 2022/12/15
Y1 - 2022/12/15
N2 - 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.
AB - 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.
KW - extraskeletal myxoid chondrosarcoma
KW - outcomes
KW - prognosis
KW - soft tissue sarcoma
KW - survival
UR - http://www.scopus.com/inward/record.url?scp=85135798396&partnerID=8YFLogxK
U2 - 10.1002/jso.27062
DO - 10.1002/jso.27062
M3 - Article
C2 - 35962783
AN - SCOPUS:85135798396
SN - 0022-4790
VL - 126
SP - 1533
EP - 1542
JO - Journal of surgical oncology
JF - Journal of surgical oncology
IS - 8
ER -