Hemangioblastomas are benign tumors of the central nervous system. They occur mostly in the posterior fossa, brain stem and spinal cord. Frequently the tumors produce a major cyst which is frequently responsible for the symptoms by a space-occupying effect. These tumors can occur sporadically or familial as a manifestation of von Hippel-Lindau (VHL) disease.
Symptoms of hemangioblastomas of the cerebellum mainly include headache, nausea, coordination difficulties, gait difficulty and dizziness. In advanced tumors, obstruction of craniospinal fluid circulation may occur, resulting in headache, nausea, vomiting and eventually loss of consciousness. If hemangioblastomas occur in the spinal cord, patients may develop pain, gait imbalance, numbness and paralysis. In addition, disturbances during defecation and urination may occur.
In general, the tumors grow slowly, so that an annual check-up by contrast enhanced MRI is sufficient to intervene in time. A microsurgical operation is the treatment of first choice. Surgical morbidity is low and tumors that have been completely removed do not recur. The effect of radiation therapy is controverse, but it may be an alternative for patients with inoperable tumors. Pharmaceutical therapies are under investigation.
Hemangioblastomas may be a component of VHL disease, a hereditary tumor syndrome. In addition to hemangioblastomas, affected patients develop retinal angiomas, renal carcinomas and adrenalin-producing tumors of the adrenal gland (pheochromocytoma). In addition, tumors in the inner ear, the epididymis and the pancreas can occur.
Patients with von Hippel-Lindau disease frequently develop multiple hemangioblastomas and therefore need a different clinical approach. Our team has special experience with hemangioblastoma patients and we are member national and international Clinical Advisory Boards for patients with this disease. Treatment decisions are discussed in our interdisciplinary treatment team.
- VHL Uz Brussel webpage http://www.uzbrussel.be/u/view/nl/10514139-von+Hippel-Lindau.html
- European VHL (von Hippel-Lindau) Federation www.vhl-europa.org
- VHL Alliance www.vhl.org