Hemangioblastoma

Hemangioblastoma, a benign vascular tumor, is seen primarily in middle-aged adults but often presents at a younger age in patients with von Hippel-Lindau disease. These tumors are primarily located in the posterior fossa, although they can occur anywhere within the neuroaxis. They are usually cystic and are sometimes classified as a variant of meningiomas (angioblastic variant). Histologically, they are characterized by distinctive vascular channels with fat-laden cells. Occasionally they can be mistaken for xanthochromic astrocytomas.^

The clinical presentation is similar to that of other posterior fossa lesions. The neuroradiological appearance is that of a well-demarcated, cystic lesion with a distinctly enhanced tumor nodule on administration of contrast material. Identification should prompt a search for other lesions including lesions of the cervical spine, especially in those with von Hippel-Lindau disease; retinal examination and evaluation for intra-abdominal neoplasms such as renal cell carcinoma or pheochromocytoma should also be done. Angiography is performed to help in this search for radiographically silent lesions and to aid in preoperative planning. Surgery is usually curative, although the use of stereotactic radiation is being explored. Because these tumors are known to express an erythropoietin-like factor, the accompanying erythrocytosis may be a marker for the extent of resection or for occult lesions in patients with known hemangioblastoma or von Hippel-Lindau disease.

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