Objectives: Extra-skeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma which accounts for less than 3% of all soft tissue tumors. It occurs most frequently in the proximal extremities of older adult males. It is characterized by its insidious growth with predilection for local recurrence and metastasis.
We presented a case of extra-skeletal myxoid chondrosarcoma which was initially misdiagnosed as May-Thurner syndrome by the cardiovascular(CV) surgeon. We searched the literature to further review the aspects of the disease, from presentation to management.
Materials and Methods: A 76-year-old male patient presented with a painless, insidious growing mass over his left calf. DVT was initially impressed but later ruled out by ultrasound. May-Thurner syndrome was suspected by the CV surgeon and initial computer computed tomography(CT) scan in 2019. However, follow-up CT scan ruled out the diagnosis three years later. MRI was arranged which suggested myxoid liposarcoma is most likely. He therefore received surgical excision for treatment and diagnosis.
Results: Pathology report revealed extra-skeletal myxoid chondrosarcoma. Follow up PET scan showed multiple nodules in bilateral lungs thus bilateral lung metastases should be suspected. There is no local recurrence during the post-op follow-up with good wound condition.
Conclusion: Extra-skeletal myxoid chondrosarcoma is rare, malignant neoplasms. We presented a patient who was initially misdiagnosed for which he received surgical excision and presented with good cosmetic outcome.

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