title: Primary diffuse leptomeningeal gliosarcomatosis.
abstract: Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition with a fatal outcome, characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. In particular, PDLG histologically diagnosed as gliosarcoma is extremely rare, with only 2 cases reported to date. We report a case of primary diffuse leptomeningeal gliosarcomatosis. A 68-year-old man presented with fever, chilling, headache, and a brief episode of mental deterioration. Initial T1-weighted post-contrast brain magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement without a definite intraparenchymal lesion. Based on clinical and imaging findings, antiviral treatment was initiated. Despite the treatment, the patient's neurologic symptoms and mental status progressively deteriorated and follow-up MRI showed rapid progression of the disease. A meningeal biopsy revealed gliosarcoma and was conclusive for the diagnosis of primary diffuse leptomeningeal gliosarcomatosis. We suggest the inclusion of PDLG in the potential differential diagnosis of patients who present with nonspecific neurologic symptoms in the presence of leptomeningeal involvement on MRI.
authors.affiliation: Department of Neurosurgery, Yonsei University Health System, Seoul, Korea. ; Brain Tumor Center, Yonsei University Health System, Seoul, Korea.
authors.forename: Ju Hyung
authors.initials: JH
authors.lastname: Moon
authors.affiliation: Brain Tumor Center, Yonsei University Health System, Seoul, Korea. ; Department of Pathology, Yonsei University Health System, Seoul, Korea. ; Brain Research Institute, Yonsei University Health System, Seoul, Korea.
authors.forename: Se Hoon
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Neurosurgery, Yonsei University Health System, Seoul, Korea. ; Brain Tumor Center, Yonsei University Health System, Seoul, Korea. ; Brain Research Institute, Yonsei University Health System, Seoul, Korea.
authors.forename: Eui Hyun
authors.initials: EH
authors.lastname: Kim
authors.affiliation: Department of Neurosurgery, Yonsei University Health System, Seoul, Korea. ; Brain Tumor Center, Yonsei University Health System, Seoul, Korea. ; Brain Research Institute, Yonsei University Health System, Seoul, Korea.
authors.forename: Seok-Gu
authors.initials: SG
authors.lastname: Kang
authors.affiliation: Department of Neurosurgery, Yonsei University Health System, Seoul, Korea. ; Brain Tumor Center, Yonsei University Health System, Seoul, Korea. ; Brain Research Institute, Yonsei University Health System, Seoul, Korea.
authors.forename: Jong Hee
authors.initials: JH
authors.lastname: Chang
keywords: Glioma, gliosarcoma; Leptomeningeal carcinomatosis; Meningoencephalitis