TY - JOUR
T1 - Long-term Outcomes for Extraskeletal Myxoid Chondrosarcoma
T2 - A SEER Database Analysis
AU - Wagner, Michael J.
AU - Chau, Bonny
AU - Loggers, Elizabeth T.
AU - Pollack, Seth M.
AU - Kim, Teresa S.
AU - Kim, Edward Y.
AU - Thompson, Matthew J.
AU - Harwood, Jared L.
AU - Cranmer, Lee D.
N1 - Funding Information:
M.J. Wagner reports personal fees and other from Deciphera (clinical trial support to institution), Tempus (clinical trial support to institution), and Adaptimmune (clinical trial support to institution) and other from Incyte (clinical trial support to institution), Athenex (clinical trial support to institution), and GlaxoSmithKline (clinical trial support to institution) outside the submitted work. S.M. Pollack reports grants from Merck, EMD Serono, Incyte, Presage, Janssen, Oncosec, and Juno and personal fees from GlaxoSmithKline, Eli Lilly, Seattle Genetics, Bayer, Tempus, Daiichi Sankyo, and Blueprint Medicine outside the submitted work. L.D. Cranmer reports grants from Eli Lilly (to institution), CBA Pharma (to institution), AdvenChen (to institution), Tracon (to institution), AADi (to institution), Exelixis (to institution), Philogen (to institution), and Iterion (to institution); grants and personal fees from BluePrint Medicines (grant to institution and honoraria); and personal fees from Daiichi Sankyo (honoraria) and Regeneron (honoraria) outside the submitted work. No potential conflicts of inte.rest were disclosed by the other authors.
Funding Information:
Anticipated long-term outcomes with current therapies must be characterized to serve as benchmarks for future studies of new therapies. Here, we assess the Surveillance, Epidemiology, and End Results (SEER) database to describe long-term OS outcomes in patients with EMCS. SEER is a cancer registry funded by the National Cancer Institute. It includes cases representing approximately one third of the U.S. population. Data is derived from cancer registries based at cancer treatment facilities across the U.S. SEER data are deidentified and publicly accessible (12, 13). This analysis can guide clinical discussions regarding prognosis and long-term outcomes for patients with EMCS and serve as a benchmark for survival in future interventional studies in this rare histology.
Publisher Copyright:
© 2020 American Association for Cancer Research.
PY - 2020/11/1
Y1 - 2020/11/1
N2 - Background: Extraskeletal myxoid chondrosarcoma (EMCS) is a rare tumor that typically has an indolent course but high rate of recurrence. We queried the Surveillance, Epidemiology, and End Results (SEER) database to assess factors associated with metastasis, treatment, and survival. Methods: We queried the SEER 1973–2016 database for patients with myxoid chondrosarcoma (ICD-O-3: 9231/3). Kaplan–Meier analyses and Cox proportional hazard models assessed effects on overall survival (OS) of demographics and clinical characteristics. Logistic regression assessed associations between tumor location and distant disease. Primary analysis was a complete case analysis; multiple imputation (MI) was used in a sensitivity analysis. Results: Locoregional disease (LRD) was found in 373 (85%) of patients. In univariate analysis with LRD, surgery correlated with superior OS [HR = 0.27; 95% confidence interval (CI), 0.16–0.47]; chemotherapy and radiotherapy associated with inferior OS (HR = 1.90; 95% CI, 1.11–3.27 and HR = 1.45; 95% CI, 1.03–2.06, respectively). No treatment modality associated with OS in the adjusted, complete case model. In the adjusted sensitivity analysis, surgery associated with superior outcomes (HR = 0.36; 95% CI, 0.19–0.69). There was no OS difference by primary tumor site. 10-year OS with distant disease was 10% (95% CI, 2%–25%). Conclusions: Surgery in LRD associated with improved OS in univariate analysis and adjusted models correcting for missing data. There was no OS benefit with chemotherapy or radiotherapy. Impact: This represents the largest report of EMCS with long-term follow-up. Despite the reputedly indolent nature of EMCS, outcomes with metastatic disease are poor. We provide OS benchmarks and guidance for stratification in future prospective trials.
AB - Background: Extraskeletal myxoid chondrosarcoma (EMCS) is a rare tumor that typically has an indolent course but high rate of recurrence. We queried the Surveillance, Epidemiology, and End Results (SEER) database to assess factors associated with metastasis, treatment, and survival. Methods: We queried the SEER 1973–2016 database for patients with myxoid chondrosarcoma (ICD-O-3: 9231/3). Kaplan–Meier analyses and Cox proportional hazard models assessed effects on overall survival (OS) of demographics and clinical characteristics. Logistic regression assessed associations between tumor location and distant disease. Primary analysis was a complete case analysis; multiple imputation (MI) was used in a sensitivity analysis. Results: Locoregional disease (LRD) was found in 373 (85%) of patients. In univariate analysis with LRD, surgery correlated with superior OS [HR = 0.27; 95% confidence interval (CI), 0.16–0.47]; chemotherapy and radiotherapy associated with inferior OS (HR = 1.90; 95% CI, 1.11–3.27 and HR = 1.45; 95% CI, 1.03–2.06, respectively). No treatment modality associated with OS in the adjusted, complete case model. In the adjusted sensitivity analysis, surgery associated with superior outcomes (HR = 0.36; 95% CI, 0.19–0.69). There was no OS difference by primary tumor site. 10-year OS with distant disease was 10% (95% CI, 2%–25%). Conclusions: Surgery in LRD associated with improved OS in univariate analysis and adjusted models correcting for missing data. There was no OS benefit with chemotherapy or radiotherapy. Impact: This represents the largest report of EMCS with long-term follow-up. Despite the reputedly indolent nature of EMCS, outcomes with metastatic disease are poor. We provide OS benchmarks and guidance for stratification in future prospective trials.
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U2 - 10.1158/1055-9965.EPI-20-0447
DO - 10.1158/1055-9965.EPI-20-0447
M3 - Article
C2 - 32856598
AN - SCOPUS:85106380702
SN - 1055-9965
VL - 29
SP - 2351
EP - 2357
JO - Cancer Epidemiology Biomarkers and Prevention
JF - Cancer Epidemiology Biomarkers and Prevention
IS - 11
ER -