gms | German Medical Science

56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)
3èmes journées françaises de Neurochirurgie (SFNC)

Deutsche Gesellschaft für Neurochirurgie e. V.
Société Française de Neurochirurgie

07. bis 11.05.2005, Strasbourg

Primarily disseminated leptomeningeal gliomatosis in a child: diagnostical and therapeutical considerations

Primär disseminierte leptomeningeale Gliomatosis bei einem Kind: diagnostische und therapeutische Aspekte

Meeting Abstract

  • corresponding author L. Dörner - Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • M. Fritsch - Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • A. Claviez - Klinik für Allgemeine Pädiatrie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • H. M. Mehdorn - Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel

Deutsche Gesellschaft für Neurochirurgie. Société Française de Neurochirurgie. 56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3èmes journées françaises de Neurochirurgie (SFNC). Strasbourg, 07.-11.05.2005. Düsseldorf, Köln: German Medical Science; 2005. DocP138

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/dgnc2005/05dgnc0406.shtml

Published: May 4, 2005

© 2005 Dörner et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Objective

Primarily disseminated leptomeningeal gliomatosis (PDLG) is rarely diagnosed tumor and often only proven postmortem. We present a case of a girl with an internal hydrocephalus. During the following diagnostic routine a PDLG was identified. The diagnostic and therapeutic difficulties with PDLG are presented here.

Methods

A 2 years old girl was admitted with somnolence, intermittent diplopia and vomiting. CCT and cMRI showed an internal hydrocephalus with an enhanced basal leptomeninx. The liquor demonstrated a mild pleocytosis (max. 55/µl) with elevated protein levels (max. 241 mg/dl) but no tumor cells. An infection with mycobacteria was excluded. As the symptomatic worsened a ventricular drainage and a consecutive dural and leptomeningeal biopsy in the sylvian fissure were performed. The biopsy showed only unspecific signs of a meningeal infection. In the further course of the disease the girl developed progressive radicular predominantly lumbar symptoms with paresthesia and hyperalgesia. A spinal MRI showed a massive contrast enhancing thickening of the spinal dura. In a spinal biopsy finally a paucicellular astrocytic differentiated tumor (WHO grade I) was diagnosed. In the immunohistochemistry the tumor proved positive for CD68 and GFAP. The proliferation index with Ki-67 was <5%.

The hydrocephalus was treated with a ventriculo-peritoneal shunt, the PDLG according to the multicenter therapy trial for low grade gliomas (SIOP-LGG 2004) with vincristine, carboplatin and etoposide. So far a tumor remission has been achieved lasting 14 months.

Results

PDLG is a rare cause of hydrocephalus. The radiological and laboratory diagnosis is difficult to establish. This case shows that even with negative liquor cytology and unspecific changes in biopsy a PDLG can not be excluded. Therapy for the hydrocephalus is a liquor shunt. The tumor therapy is far more difficult, because surgery is not an option and tumors are often of low grade. But not only in these patients is the response to chemotherapy not encouraging.

Conclusions

The diagnosis of PDLG can be a challenge not only because of their paucity. There is no standard tumor therapy so far and the published results are not promising. Further data about PDLG needs to be gathered.