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Cerebellar haemangioblastoma

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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A haemangioblastoma is a tumour of vascular origin which occurs primarily in young and middle aged adults, slightly more often in males. Typically, it presents as a cystic swelling in one or more cerebellar hemispheres, but it may present in the retina, spinal cord and other sites. Occasionally, they may be multiple. They may be associated with other pathologies such as polycythaemia and cystic lesions in other tissues such as in von Hippel Lindau disease.

Presentation is characteristically one of a rapid evolution of cerebellar disturbance with raised intracranial pressure. Subarachnoid haemorrhage may occur. In females, onset may be coincident with pregnancy. The tumour is revealed on CT as a well defined, low density cystic lesion with a strong enhancing nodule in the wall. Angiography helps differentiate multiple tumours from multiple metastases.

Treatment is by excision. The recurrence rate is 20%.

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