Low-grade fibromyxoid sarcoma of intracranial origin

The authors report on a 21-year-old man who presented with a low-grade fibromyxoid sarcoma primarily located in the right parietal lobe with diffuse infiltration. The low-grade fibromyxoid sarcoma is a rare sarcoma of the deep soft tissue that is characterized as an indolent but metastasizing soft-tissue neoplasm with a deceptively benign histological appearance. Only one case of intracranial origin has been previously reported in the literature. A high rate of local recurrence and eventual metastasis has been demonstrated for this tumor in deep soft tissue. Similarly, the patient in the present case suffered recurrence 6 times; he underwent treatment by surgical removal 4 times, Gamma Knife surgery twice, and local radiation therapy once during the 7-year follow-up period. The tumor is still under control without any evidence of extracranial metastasis. To the authors' knowledge, this is the first case report that discusses the clinical course of this rare disease in detail.