Turkish Neurosurgery 2015 , Vol 25 , Num 6
Malignant Peripheral Nerve Sheath Tumor Manifesting as Severe Buttock Pain
Satoshi TSUTSUMI1, Masanori ITO1, Ikuko OGINO3, Hiroshi IZUMI2, Hajime ARAI3, Yukimasa YASUMOTO1
1Juntendo University, Urayasu Hospital, Department of Neurological Surgery, Urayasu, Chiba, Japan
2Juntendo University, Urayasu Hospital, Department of Pathology, Urayasu, Chiba, Japan
3Juntendo University, School of Medicine, Department of Neurological Surgery,Tokyo, Japan
DOI : 10.5137/1019-5149.JTN.12046-14.1 A 28-year-old woman presented with complaints of severe buttock pain exacerbating for 2 weeks. Physical examination found numerous caf-au-lait macules and axillary freckles. Neurological examination revealed sensory loss at the S3-S5 dermatome and significant vesicorectal dysfunction. Magnetic resonance imaging revealed an enhanced intraspinal mass at S1-S2, with bony erosion on the dorsal aspect of the sacrum. Intraoperatively, the rostral part of the tumor was found to involve a filament of the cauda equina. The tumor protruded extraspinally through an irregular-shaped defect in the dorsal dura and bony erosion. Total resection was achieved. The histological appearance was consistent with malignant peripheral nerve sheath tumor and loss of neurofibromin. Malignant peripheral nerve sheath tumor should be included in the differential diagnosis of spinal tumor in a patient with neurofibromatosis type 1. Keywords : Malignant peripheral nerve sheath tumor, Dysuria, Neurofibromatosis type 1
Corresponding author : Satoshi Tsutsum, shotaro@juntendo-urayasu.jp