Article
Case report – a space occupying brain lesion mimickring a high grade glioma emerging as a case of the rare hereditary cerebroretinal vasculopathy
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Authors
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Martin Reiser
- Klinik für Neurochirurgie, Akademisches Lehrkrankenhaus München Bogenhausen der Technischen Universität München, Deutschland
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Reinhold Winkler
- Klinik für Neurochirurgie, Akademisches Lehrkrankenhaus München Bogenhausen der Technischen Universität München, Deutschland
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Christianto Lumenta
- Klinik für Neurochirurgie, Akademisches Lehrkrankenhaus München Bogenhausen der Technischen Universität München, Deutschland
| Published: | September 16, 2010 |
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Outline
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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.
