gms | German Medical Science

61st Annual Meeting of the German Society of Neurosurgery (DGNC) as part of the Neurowoche 2010
Joint Meeting with the Brazilian Society of Neurosurgery on the 20 September 2010

German Society of Neurosurgery (DGNC)

21 - 25 September 2010, Mannheim

Case report – a space occupying brain lesion mimickring a high grade glioma emerging as a case of the rare hereditary cerebroretinal vasculopathy

Meeting Abstract

Search Medline for

  • Martin Reiser - Klinik für Neurochirurgie, Akademisches Lehrkrankenhaus München Bogenhausen der Technischen Universität München, Deutschland
  • Reinhold Winkler - Klinik für Neurochirurgie, Akademisches Lehrkrankenhaus München Bogenhausen der Technischen Universität München, Deutschland
  • Christianto Lumenta - Klinik für Neurochirurgie, Akademisches Lehrkrankenhaus München Bogenhausen der Technischen Universität München, Deutschland

Deutsche Gesellschaft für Neurochirurgie. 61. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC) im Rahmen der Neurowoche 2010. Mannheim, 21.-25.09.2010. Düsseldorf: German Medical Science GMS Publishing House; 2010. DocP1852

DOI: 10.3205/10dgnc323, URN: urn:nbn:de:0183-10dgnc3237

Published: September 16, 2010

© 2010 Reiser 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: We report a case of a 47-year-old man with suspected space occupying brain tumor turning out as a case of hereditary cerebroretinal vasculopathy (CRV), first described by Grand et al. and confirmed by Niedermayer et al. and Weil et al. ..

Methods: We treated a 47-year-old man because of a hemiparesis increasing over a period of 2 years and a recent series of complex partial seizures. As family history refered, his brother and two cousins died from “brain tumors”. The full orientatet patient featured a spastic hemiparesis on the right and a severe aphasia. CCT showed a right frontal lesion with extensive edema and resulting significant brain shift. At subsequent MRI it presented with contrast enhancement suggesting a glioblastoma multiforme in differential diagnosis. We first treated the patient with dexamethason. The ophthalmologic examination showed no morphologic pathologic results. Cerebral DSA only accounted for indirect displacement of cerebral vessels without malformation. We decided to operate on the patient for in the manner of a extended microsurgical biopsy to reduce space occupying effects and to clarify the histopathologic entity.

Results: The postsurgical MRI showed almost no brainshift. Our histopathologic analysis was reminiscent of delayed radiation-induced coagulation brain tissue necrosis, formations of modified multilayerd basement membranes. This results were approved by two reference centers and assorted completely with the findings published about other cases of this rare hereditary syndrome. The furthermore implemented fluorescein angiography of small retinal vessels showed typical microvasular changes with microaneurysms. The residual cerebral lesion did not present a significant tracer uptake in the additional performed brain PET study. At the time of discharge from hospital the neurological deficits, particularly aphasia, were considerably improved.

Conclusions: In patients with a positive family history of fatal “brain tumors”, a space occupying intracerebral lesion with MRI contrast enhancement suggesting a high glioma with no significant traceruptake in brain PET, CRV should be considered as a differential diagnosis and a fluorescein angiography of small retinal vessels should be induced for approval.