J Clin Neurol.  2006 Sep;2(3):202-205. 10.3988/jcn.2006.2.3.202.

Primary Diffuse Leptomeningeal Gliomatosis: Report of a Case Presenting with Chronic Meningitis

• Kim SH
• Jun DC
• Park JS
• Heo JH
• Kim SM
• Kim J
• Paek SH
• Kim M

• KMID: 2287695
• DOI: http://doi.org/10.3988/jcn.2006.2.3.202

Abstract

Neoplastic meningitis occurs in approximately 5% of patients with cancer. Primary diffuse leptomeningeal gliomatosis is a rare condition whereby a glioma arises from heterotopic cell nests in the leptomeninges. We report here a case presenting with clinical features similar to those of chronic infectious meningitis without positive cerebrospinal fluid cytology. Neurological signs in our patient deteriorated progressively without responding to antitubercular, antiviral, or antibiotic therapy. Leptomeningeal biopsy sampling revealed the condition to be primary diffuse leptomeningeal gliomatosis.

Keyword

Primary diffuse leptomeningeal gliomatosis; Chronic meningitis

MeSH Terms

Biopsy
Cerebrospinal Fluid
Glioma
Humans
Meningitis*

Figure

• 

  Figure 1 T1-weighted axial and sagittal brain MRI. There was extensive hydrocephalus and marked thickening of the meninges with prominent gadolinium enhancement, and a leptomeningeal-enhancing nodular mass above the corpus callosum.

• 

  Figure 2 Biopsy of the leptomeningeal mass above the corpus callosum. (A) Hematoxylin-eosin-stained meningeal tumor with marked hypercellularity, nuclear pleomorphism, geographic pseudopalisading necrosis, and venous endothelial hyperplasia (×100). (B) Positive glial fibrillary acidic protein staining of the tumor cells (×200).

Reference

1. Chamberlain MC. Neoplastic meningitis. Semin Neurol. 2004. 24:363–374.

2. Cooper IS, Kernohan JW. Heterotropic glial nests in subarachnoid space; histopathologic characteristics, mode of origin and relation to meningeal gliomas. J Neuropathol Exp Neurol. 1951. 10:16–29.

3. Riva M, Bacigaluppi S, Galli C, Citterio A, Collice M. Primary leptomeningeal gliomatosis: case report and review of the literature. Neurol Sci. 2005. 26:129–134.

4. Kastenbauer S, Danek A, Klein W, Yousry TA, Bise K, Reifenberger G, et al. Primary diffuse leptomeningeal gliomatosis: unusual MRI with non-enhancing nodular lesions on the cerebellar surface and spinal leptomeningeal enhancement. J Neurol Neurosurg Psychiatry. 2000. 69:385–388.

5. Debono B, Derrey S, Rabehenoina C, Proust F, Freger P, Laquerriere A. Primary diffuse multinodular leptomeningeal gliomatosis: case report and review of the literature. Surg Neurol. 2006. 65:273–282.