A case of primary diffuse leptomeningeal gliomatosis

Kouji Yamasaki1, Kiyotaka Yokogami1, Hajime Ohta2, Shinji Yamashita1, Hisao Uehara1, Yuichiro Sato3, Hideo Takeshima1
1Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan
2Department of Neurosurgery, Miyakonojo Medical Association Hospital, Miyazaki, Japan
3Department of Pathology, Faculty of Medicine, University Miyazaki, Miyazaki, Japan

Tóm tắt

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare and fatal disease characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of tumor in the brain parenchyma or spinal cord. We report a 60-year-old man with PDLG. He suffered transient right hemiparesis and generalized seizures. MRI showed diffuse leptomeningeal thickening and enhancement throughout the brain and spinal cord without any intraaxial involvement. Biopsy resulted in a diagnosis of glioblastoma with methylated MGMT promoter and wild-type IDH1. He underwent craniospinal radiotherapy and temozolomide treatment but despite concomitant adjuvant therapy he died 8 months after initial presentation.

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Tài liệu tham khảo

Arita N, Taneda M, Hayakawa T (1994) Leptomeningeal dissemination of malignant gliomas: incidence, diagnosis and outcome. Acta Neurochir (Wien) 126:84–92 Erlich SS, Davis RL (1978) Spinal subarachnoid metastasis from primary intracranial glioblastoma multiforme. Cancer 42:2854–2864 Onda K, Tanaka R, Takahashi H et al (1989) Cerebral glioblastoma with cerebrospinal fluid dissemination: a clinicopathological study of 14 cases examined by complete autopsy. Neurosurgery 25:533–540 Parsa AT, Wachhorst S, Lamborn KR et al (2005) Prognostic significance of intracranial dissemination of glioblastoma multiforme in adults. J Neurosurg 102:622–628 Esteller M, Garcia-Foncillas J, Andion E et al (2000) Inactivation of the DNA-repair gene MGMT and the clinical response of gliomas to alkylating agents. N Engl J Med 343:1350–1354 Cooper IS, Kernohan JW (1951) Heterotropic glial nests in the subarachnoid space: histopathologic characteristics, mode of origin and relation to meningeal gliomas. J Neuropathol Exp Neurol 10:16–29 Yomo S, Tada T, Hirayama S et al (2007) A case report and review of the literature. J Neurooncol 81:209–216 Baborie A, Dunn EM, Bridges LR et al (2001) Primary diffuse leptomeningeal gliomatosis predominantly affecting the spinal cord: case report and review of the literature. J Neurol Neurosurg Psychiatry 70:256–258 Corsten LA, Raja AI, Wagner FC Jr (2001) Primary diffuse leptomeningeal gliomatosis. Br J Neurosurg 15:62–66 Arita H, Narita Y, Fukushima S et al (2013) Upregulating mutations in the TERT promoter commonly occur in adult malignant gliomas and are strongly associated with total 1p19q loss. Acta Neuropathol 126:267–276 Killela PJ, Reitman ZJ, Jiao Y et al (2013) TERT promoter mutations occur frequently in gliomas and a subset of tumors derived from cells with low rates of self-renewal. Proc Natl Acad Sci USA 110(15):6021–6026 Nonoguchi N, Ohta T, Oh JE et al (2013) TERT promoter mutations in primary and secondary glioblastomas. Acta Neuropathol 126:931–937 Bohner G, Masuhr F, Distl R (2005) Pilocytic astrocytoma presenting as primary diffuse leptomeningeal gliomatosis: report of a unique case and review of the literature. Acta Neuropathol (Berl) 110(3):306–311 Izumoto S, Ohnishi T, Kenemura H (2001) PTEN mutations in malignant gliomas and their relation with meningeal gliomatosis. J Neurooncol 53(1):21–26 Franceschi E, Cavallo G, Scopece L (2005) Temozolomide-induced partial response in a patient with primary diffuse leptomeningeal gliomatosis. J Neurooncol 73(3):261–264 Hansen N, Wittig A, Hense J (2011) Long survival of primary diffuse leptomeningeal gliomatosis following radiotherapy and temozolomide: case report and literature review. Eur J Med Res 16(9):415–419 Stupp R, Mason WP, van den Bent MJ et al (2005) Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med 352:987–996