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Clinical Neurology and Neurosurgery 2010-Oct

Transformation of intracranial anaplastic astrocytoma associated with neurofibromatosis type I into gliosarcoma: case report.

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Jin-Hwan Cheong
Choong-Hyun Kim
Jae-Min Kim
Young-Ha Oh

Mots clés

Abstrait

Gliosarcoma is an uncommon malignant brain tumor composed of distinct sarcomatous and malignant glial cell elements. These tumors are defined as a variant of glioblastoma, and it can be developed by progression of the malignant glial cell tumors or primary tumors. We report a rare case with gliosarcomatous recurrence of anaplastic astrocytoma with neurofibromatosis type 1 (NF-1) followed by chemoradiation therapy. A 26-year-old male patient was presented with headache. Five years before admission, he had been diagnosed with NF-1. Magnetic resonance imaging (MRI) showed a well-demarcated, enhanced lesion in the right frontal lobe and multiple enhanced lesions in the scalp, lower cervical, thoracic, and upper lumbar regions. He underwent an osteoplastic craniotomy with total tumor resection. Histopathology of the tumor showed findings corresponding with anaplastic astrocytoma. He was followed by radiotherapy and chemotherapy postoperatively. A month later, his spinal lesion was also resected and confirmed pathologically as plexiform neurofibroma. The subsequent follow-up period of 27 months was uneventful until he developed a generalized tonic-clonic seizure. MRI showed tumor recurrence in the original site of the tumor. Re-exploration was carried out. Pathological examination displayed a biphasic pattern of the glial and sarcomatous components suggesting gliosarcoma.

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