Haemangioblastoma, a vascular neoplasm within the parenchyma of the neuraxis, accounts for approximately 2 per cent of all intracranial neoplasms. This tumour is most common in middle-aged adults, with a peak incidence in the fourth decade. Men are affected more commonly than women.

Haemangioblastomas occur most commonly within the cerebellum, where the typical appearance is that of a cystic tumour. The neoplastic tissue is present as a nodule in the wall of the cyst. Haemangioblastomas may also present as solid tumours. The second most common site of occurrence is within the spinal cord, where they usually appear as a cystic intramedullary lesion. Within the spinal cord these tumours usually derive their major blood supply from the anterior spinal artery. Very rarely haemangioblastomas occur in the cerebral hemispheres or in the nerves or nerve roots. Histologically, these tumours are considered to be benign; therefore complete surgical resection is usually curative. Incomplete resections are associated with continued growth of the residual tumour.

Haemangioblastomas may occur sporadically as isolated tumours or they may be part of von Hippel Lindau's complex, an inherited disorder characterized by multiple haemangioblastomas in the neuraxis, as well as visceral disorders, including retinal angiomatosis, renal cell carcinoma, renal cysts, pancreatic cysts, and phaeochromocytomas. The von Hippel Lindau complex is transmitted as an autosomal dominant disorder with variable penetrance. These patients need to be monitored carefully for the development of new haemangioblastomas.

The signs and symptoms of haemangioblastoma are dependent on the location of the tumour. Cerebellar haemangioblastoma causes headache, nausea, vomiting, stiff neck, and diplopia. Haemangioblastomas within the spinal cord produce progressive myelopathy. Polycythaemia may be seen in up to 20 per cent of patients with haemangioblastoma.

Patients in whom haemangioblastoma is suspected are best evaluated with high resolution MRI scanning, which show the cystic component of the tumour. The neural nodule characteristically enhances with gadolinium infusion.

Haemangioblastomas are managed best by complete surgical excision. The cystic cerebellar haemangioblastoma is comprised of a large cyst and small neural nodule: the latter is the neoplasm that must be resected. This neural nodule will usually be supplied by one or more sizable arteries. Adjunctive therapy is not required.