Korea Institute of Radiological Medical Sciences Seoul, Seoul-t'ukpyolsi, Republic of Korea
Objective: Extraskeletal myxoid chondrosarcoma (EMC) is an extremely rare malignant mesenchymal neoplasm. accounting for less than 3% of soft tissue sarcoma. This sarcoma is usually characterized by an indolent course. The aim of our study was to document the clinical manifestation and oncologic outcomes of EMC.
Methods: This study identified 17 patients who were diagnosed and treated for EMC between January 2008 and December 2018. The identified cohort was then reviewed regarding age, gender, symptom onset, tumor location, MR images, surgical margin and pathologic diagnosis. The time to local recurrence and metastasis, follow-up duration, and the patients’ final status were analyzed.
Results: The patients comprised 10 males and 7 female patients with a mean age of 54 (range, 31-79). The location of the tumor was buttock in 5, knee in 3, foot in 2, shoulder in 1, and back in 1. The average tumor diameter was 11.5 cm (range, 6-26 cm). At the time of diagnosis, 5 patients were AJCC stage II, 3 were IIIA, 3 were IIIB, and 6 were IV. Local recurrence occurred in 12 cases and distant metastasis occurred in 15 cases. The 5-year overall survival of patients with EMC was 73 ± 17%, and 2 patients died of the disease.
Conclusion: In spite of a high rate of local recurrence and distant metastasis, long-term survival rate in patients with EMC is quite high because of its indolent characteristics.
