spontaneous regression of two putative supratentorial haemangioblastomas in one patient

Case Details

Disease Location

Trigone, occipital and frontal locations

Personal Characteristics

61 -year-old male, admitted because he had a generalized seizure

Clinical Characteristics

3 supratentorial haemangioblastomas discovered via contrast enhancing MRI, each lesion was approx. 2 cm in the largest diameter one was located frontal near the motor area, one in trigone area, and another in the occipital lobe tumor appeared reddish and was richly vascularized with several supplying arteries, and the tumor had no relationship with the dura. The tumor cells are postive for neuron specific enolase and negative for epithelial membrain antigen supratentorial haemanioblastomas are rare, are sporadic or associated with von hippel-lindau disease abdominal CT scan for von hippel lindau disease, ophthalmologic evaluation, and mutations of the vhl gene were all negative histopathological and immunohistochemical findings of excised tumor were consistent with hemoangioblastoma (details in article) which are histologically benign

Remission Characteristics

Upon the removal of one lesion (occipital hemoangioblastoma), the other two disappeared MRI follow-ups 6 months and 1 year after surgery confirmed remission of frontal and trigone lesions both follow-ups there remained a small residue of hemosiderin corresponding to the location of the frontal lesion

Treatment & Mechanisms

Proposed Remission Mechanisms

The removed tumor was supporighting the existence of the other two through trophic factors the vhl gene, by negative regulation of vascular endothelial growth factor (vegf) has been seen to control the angiogenesis of tumor cells and strong vegf has been shown in haemangioblastomas vegf is involved in endothelial proliferation in early steps of angiogenesis

Clinical Treatment

Craniotomy of occipital lesion only, because of potential consequences

Non-Clinical Treatment

None reported