Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy

Drilon, A. D., Popat, S., Bhuchar, G., D'Adamo, D. R., Keohan, M. L., Fisher, C., Antonescu, C. R., Singer, S., Brennan, M. F., Judson, I., Maki, R. G. (December 2008) Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Cancer, 113 (12). pp. 3364-71. ISSN 0008-543X (Print)0008-543X (Linking)

URL: https://www.ncbi.nlm.nih.gov/pubmed/18951519
DOI: 10.1002/cncr.23978

Abstract

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a genetically distinct sarcoma with a propensity for local recurrence and metastasis despite an indolent course. To the authors' knowledge, there are limited data examining chemotherapy outcomes as a guide to therapeutic decisions for unresectable disease. METHODS: The clinical behavior and treatment responses of 87 patients with EMC who were seen at 2 institutions between 1975 and 2008 were examined. RESULTS: The median age of the patients at the time of diagnosis was 49.5 years, with a male-to-female ratio of 2:1. For patients presenting without metastases, 37% developed local recurrence (median time of 3.3 years) and 26% developed distal recurrence (median time of 3.2 years). Approximately 13% of patients presented with metastases. The 5-year, 10-year, and 15-year overall survival rates were 82%, 65%, and 58%, respectively. Twenty-one patients received 32 evaluable courses of chemotherapy. No significant radiologic or clinical responses were noted. The median time to disease progression while receiving chemotherapy was 5.2 months. The best physician-assessed response to chemotherapy was stable disease for at least 6 months in 25% of patients, stable disease for <6 months in 41% of patients, and disease progression in 34% of patients. The estimated progression-free survival rates at 3 months, 4 months, 6 months, and 9 months were 69%, 65%, 40%, and 26%, respectively. CONCLUSIONS: This retrospective review highlights the poor response rate to chemotherapy and emphasizes aggressive control of localized disease as the primary approach to management. Although there are biases inherent in retrospective analyses, these data provide a benchmark for time to disease progression for the study of new agents for the treatment of patients with this diagnosis.

Item Type: Paper
Uncontrolled Keywords: Adolescent Adult Aged Aged, 80 and over Child Chondrosarcoma/*drug therapy/*surgery Disease-Free Survival Female Humans Male Middle Aged Retrospective Studies Soft Tissue Neoplasms/*drug therapy/*surgery Survival Analysis Treatment Outcome
Subjects: diseases & disorders > cancer > drugs and therapies > chemotherapy
diseases & disorders > cancer > prognosis
diseases & disorders > cancer > cancer types > sarcoma
CSHL Authors:
Communities: CSHL labs > Maki lab
Depositing User: Matt Covey
Date: 15 December 2008
Date Deposited: 25 Oct 2016 20:55
Last Modified: 25 Oct 2016 20:55
PMCID: PMC2779719
Related URLs:
URI: http://repository.cshl.edu/id/eprint/33687

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