Overview
These relatively uncommon benign tumors are comprised of blood vessels and typically arise in the cerebellum, but can also occur in the brainstem and spinal cord. They typically have a solid and a cystic component. Hemangioblastomas may also occur in patients with Von Hippel-Lindau disease, an inherited autosomal dominant condition in which both benign and malignant tumors develop including retinal angiomas, pancreatic cysts, pheochromocytoma and renal cell carcinoma.
Symptoms
Patients with a cerebellar hemangioblastoma typically develop symptoms related to the enlarging tumor and cyst putting pressure on the cerebellum or causing hydrocephalus (excess cerebrospinal fluid). Common symptoms include headache, loss of coordination, imbalance, nausea and vomiting. Brainstem and spinal cord hemangioblastomas may cause loss of motor function (weakness) and loss of sensation in the extremities or impaired bowel and bladder function.
Diagnosis
Hemangioblastomas are typically diagnosed by MRI of the brain or spinal cord. A CT scan of the brain will also identify most cerebellar and brainstem hemangioblastomas. The solid part of the hemangioblastoma (mural nodule) enhances intensely after contrast administration on both MRI and CT while the associated cyst (fluid) does not enhance.
Treatment
Symptomatic cerebellar or brainstem hemangioblastomas are typically treated by surgical removal through a sub-occipital or Retromastoid Craniotomy. The goal of surgery is to remove the enhancing tumor nodule completely which is able to be accomplished in 80-90% of patients. For incompletely removed tumors, radiosurgery or stereotactic radiotherapy is relatively effective. Any patient diagnosed with a hemangioblastoma should have a thorough evaluation for Von Hippel-Lindau disease.