Hemangioblastomas are uncommon, slow-growing tumors of the central nervous system, which most commonly occur in the cerebellum, brainstem, or spinal cord. They account for approximately 4 percent of all spinal cord tumors, and 7 to 10 percent of tumors arising in the posterior fossa in adults.
Hemangioblastomas occur both sporadically and as an important component of von Hippel-Lindau (VHL) disease, along with retinal angiomas, renal cell carcinoma, pheochromocytomas, pancreatic cysts and neuroendocrine tumors.
The clinical manifestations and management of patients with hemangioblastomas are reviewed here. The clinical manifestations and overall management of patients with VHL disease are discussed separately. (See "Clinical features, diagnosis, and management of von Hippel-Lindau disease".)
Sporadic versus VHL-associated tumors — Hemangioblastomas may occur either sporadically or as a manifestation of VHL disease. Although approximately 75 percent of all hemangioblastomas appear to be sporadic , some of these may represent occult cases of VHL that can be detected if patients are appropriately screened for germline VHL mutations. (See "Clinical features, diagnosis, and management of von Hippel-Lindau disease", section on 'Hemangioblastomas'.)
Sporadic and VHL-related hemangioblastomas differ significantly in their presentation: