Alerts and Notices
SynopsisA hemangioblastoma, or angioblastoma, is an uncommon benign, low-grade, highly vascular tumor originating from capillary-generating endothelial cells of the central nervous system. Common locations include the brain stem, cerebellum, and spinal cord. Approximately 1 in 4 cases occur in the context of von Hippel-Lindau disease (VHL), an autosomal dominant disorder characterized by tumors in the central nervous system, as well as renal cell carcinoma and adrenal gland and neuroendocrine tumors.
Onset is usually in adulthood and rarely in childhood. Signs and symptoms include ataxia, headache, motor and sensory deficits, nausea, vomiting, and back pain. Symptoms occur due to direct compression or hemorrhage from the tumor.
Management includes surgical excision, and the prognosis is favorable if diagnosed early.
D18.00 – Hemangioma unspecified site
81201000 – Hemangioblastoma
Differential Diagnosis & Pitfalls
- Metastatic tumor
- Primary central nervous system lymphoma
- Arteriovenous malformation
- Central neuroblastoma
- Infarction (eg, brain stem, cerebellar)
- Hemorrhage (eg, cerebellar, intracerebral)