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Journal of Neurosurgery
 
Journal of Neurosurgery: Spine
 
Journal of Neurosurgery: Pediatrics
 
Neurosurgical Focus

Low-grade fibromyxoid sarcoma of intracranial origin
Case report

Ryuta Saito, M.D.1, Toshihiro Kumabe, M.D.1, Mika Watanabe, M.D.2, Hidefumi Jokura, M.D.3, Makoto Shibuya, M.D.4, Yoichi Nakazato, M.D.5, and Teiji Tominaga, M.D.1
1Departments of Neurosurgery and 2Pathology, Tohoku University Graduate School of Medicine, Sendai; 3Department of Neurosurgery, Jiro Suzuki Memorial Gamma House, Furukawa Seiryo Hospital, Oosaki, Miyagi; 4Department of Diagnostic Pathology, Tokyo Medical University, Hachioji Medical Center, Hachioji, Tokyo; and 5Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

Abbreviations used in this paper: CT = computed tomography; EMA = epithelial membrane antigen; Gd-DTPA = gadolinium-di-ethylenetriaminepentaacetic acid; MR = magnetic resonance.

Address correspondence to: Toshihiro Kumabe, M.D., Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-8574, Japan. email: .

DOI: 10.3171/JNS/2008/108/4/0798

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.

Keywords: Gamma Knife surgery; intracranial tumor; low-grade fibromyxoid sarcoma; mesenchymal tumor; recurrence.

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