Management of Metastatic Extraskeletal Myxoid Chondrosarcoma: Role of Surgery and Chemotherapy

What is it about?

Extraskeletal myxoid chondrosarcoma (EMC) is an extremely rare soft tissue sarcoma (≤ 1/1,000,000 inhabitants/year), characterized by an indolent but metastasizing course; distant metastases (M1) occur in 30–50% of cases, predominantly to the lungs. However, there is limited research on the management of metastatic disease, particularly when metastasectomy and chemotherapy (CHT) are employed. Two patients with a histopathologically confirmed diagnosis of EMC are described and were treated between 2013 and 2024 at a reference center, who underwent metastasectomy in the course of metastatic disease. Both patients underwent radical resection of the primary tumor (R0) and perioperative radiotherapy. Pulmonary M1 were indentified 17 months and 1 month after surgery, respectively. A total of 5 metastasectomies were performed. M1 recurrences were typical — the first after approximately 3 months in patient A and between 6 and 34 months after subsequent resections in patient B, with widespread progression diagnosed 24 months after the last extrapulmonary M1 resection. In the treatment of metastatic disease, first-line CHT (ADIC regimen; agents and dosing varied by case) was used in patient B with radiological progression after 3 months, while in patient A the ADIC regimen was limited due to a high risk of cardiotoxicity — LVEF 35–40%. In the second line, GEM/DTX brought short-term stabilization in patient B; in the case of A, second-line treatment was not implemented for cardiological reasons. No lasting systemic responses were achieved. Metastasectomy in patients diagnosed with EMC allows for temporary control of the disease, however, does not prevent subsequent M1 recurrences — repeat surgical procedures are necessary. The implementation of CHT is of limited benefit and cardiac risk has to be considered. Keywords: extraskeletal myxoid chondrosarcoma, multidisciplinary treatment, preoperative radiotherapy, metastasectomy, chemotherapy

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Photo by Mario Verduzco on Unsplash

Photo by Mario Verduzco on Unsplash

Why is it important?

Extraskeletal myxoid chondrosarcoma is ultra-rare, so guidance on sequencing metastasectomy, radiotherapy and systemic therapy in metastatic disease is largely inferred from small, mixed-series data. Our case series provides detailed real-world trajectories, linking repeated pulmonary metastasectomy, perioperative radiotherapy, anthracycline- and taxane-based chemotherapy, VEGFR inhibition and cardio-oncology assessment. It offers pragmatic signals on when surgery is still worthwhile, when chemotherapy should be withheld or stopped, and when to prioritise targeted therapy or supportive care, which may help MDTs refine management where prospective trials are unlikely.

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