Intracranial Low-Grade Fibromyxoid Sarcoma with Atypical Radiology

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS), an uncommon soft tissue tumor, generally occurs in extremities and is characterized by an aggressive clinical course with frequent recurrences. Primary intracranial occurrence of LGFMS is rare. The few existing descriptions present the clinicopathologic aspects of LGFMS and lack information from a radiologic perspective. In these reports, the lesions appeared solid on imaging. CASE DESCRIPTION: In a 45-year-old woman, a falx-based lesion with solid-cystic radiologic appearance that turned out to be LGFMS on histopathology. The patient's clinical follow-up (2 years) is presented along with a literature review of other primary central nervous system cases. CONCLUSIONS: This report emphasizes the radiologic aspects of LGFMS and describes an atypical solid-cystic appearance of this uncommon central nervous system tumor. This adds another facet to the imaging of LGFMS. Although rare, LGFMS can form a differential diagnosis of dural-based lesions.