Fibromyxoid Sarcoma

Low-grade fibromyxoid sarcoma (LGFMS) is a rare, low-grade malignant neoplasm that usually develops in the proximal extremities and trunk and typically presents in younger adults. Local recurrence and metastases are common in adults and rare in children. CT, ultrasound, and MRI findings are useful in identifying tumor burden and determining the presence and extent of metastasis. Histopathologic examination and genetic/protein analysis are necessary to diagnose LGFMS. Expression of MUC4 and presence of the FUS/CREB3L2 fusion gene are key markers. Surgical resection with negative margins is the most effective way to prevent recurrence and metastasis.