Bilateral chronic subdural haematomas in a patient with meningioma of the superior sagittal sinus - case report and pathophysiological study.

Bilateral chronic subdural haemorrhage accompanying meningioma is a very rare clinical condition. We present a case of a 69-year-old female patient with large meningioma completely obliterating the posterior third part of the superior sagittal sinus with accompanying bilateral chronic subdural haematomas. Three anatomical zones of venous collateral circulation were revealed by the preoperative digital subtraction angiography. The tumour and haematomas were removed completely with no major complications. The most likely pathomechanism of the development of bilateral chronic subdural haematomas was venous hypertension caused by an occlusion of major cerebral venous trunks. As a result of a minor thrombotic incident or insignificant head injury, the distended veins of collateral circulation that were volumetrically burdened could have been damaged. Patients with large tumours occluding the superior sagittal sinus, who did not qualify for or refused surgery, should be carefully monitored clinically and neuroradiologically because of possibly increased risk of an intracranial haemorrhage.

Mutations in the von hippel-lindau tumour suppressor gene in central nervous system hemangioblastomas.

Central nervous system hemangioblastomas (cHAB) are rare tumours which most commonly arise in the cerebellum. Most tumours are sporadic, but as many as one third of cHABs occur in the course of the hereditary disorder - von Hippel-Lindau disease (VHL). In order to diagnose new VHL families in Poland we performed sequencing of the entire VHL gene in archival material (paraffin embedded hemangioblastoma tissues) in a large series of 203 unselected patients with cHAB. VHL gene mutations were detected in 70 (41%) of 171 tumour samples from which DNA of relatively good quality was isolated. We were able to obtain blood samples from 19 of mutation positive cases. Eight (42%) of these harboured germline mutations in persons from distinct undiagnosed VHL families.