Primary intracranial low-grade fibromyxoid sarcoma (Evans tumor)

Low-grade fibromyxoid sarcoma was first described in 1987 as a rare soft tissue neoplasm characterized by a bland and deceptively benign histological appearance but with aggressive behavior. A 20-year-old male patient presented with a recent history of headache and seizure. A right frontal mass was detected on MRI and he was operated upon to remove the intracranial mass. Histological examination revealed mildly atypical fibroblastic cells embedded within a myxoid matrix. Nuclear atypia and pleomorphism were minimal, and necrosis was not present. The lesion was diagnosed as a low-grade fibromyxoid sarcoma. Although primary intracranial low-grade fibromyxoid sarcoma has characteristic histological features, clinical and radiological correlation is necessary to make the correct diagnosis.