Extraskeletal myxoid chondrosarcoma

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. Cancer 2008. © 2008 American Cancer Society.

Extraskeletal myxoid chondrosarcoma with non–EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology

“…[16] In one of the larger clinicopathologic studies to date including 42 patients with EMC, the overall survival was 100% at 5 years and 69% at 10 years. [15] The correlation between histologic grade in EMC and behavior has been controversial.…”

Clinical–Pathological Conference: Case 1

“…A subgroup of patients in this study were treated by chemotherapy, but the response to chemotherapy was poor; thus, these results emphasize that adequate surgery is the primary and most important treatment modality in EMC [7]. However, complete surgical eradication of large malignancies in the head and neck region may not always be possible.…”

“…The latter is seen in the subgroup of cases with neuroendocrine differentiation. Other abnormalities such as trisomies 7,8,12,18,19, and 22, as well as structural rearrangements in 1q, 2q, 3q, 4q31, 6q have also been observed but are not diagnostic for EMC [2,4,5]. The EWSR1-NR4A3 fusion gene activates the PPARG nuclear receptor gene, and this may have clinical relevance as several PPARG agonists have been developed and are presently at various stages of clinical trials [4].…”

Extraskeletal Myxoid Chondrosarcoma with Small Bowel Metastasis Causing Bowel Obstruction

“…Other less usual sites of metastasis are intracranial [9] and pancreatic [10]. The local recurrence rate ranges between 37% and 48% and distant metastasis ranges between 26% and 46% depending on the published series [1, 2]. The EMC estimated 5-, 10-, and 15-year survival rates reported are 90%, 70%, and 60%, respectively.…”