Last Updated: September 20, 2020
- Usually benign, vascular, and slow-growing adult cerebellar tumor
- WHO grade I
- Well-circumscribed, highly vascular mass composed of stromal cells and small blood vessels
- Sporadic or familial
- Familial cases associated with von Hippel-Lindau (VHL) disease
- Up to 40% of all hemangioblastomas (HGBLs) are in patients with VHL disease
- Often multiple, including retinal HGBLs
- Sporadic and VHL disease cases both demonstrate erythropoietin upregulation
- Sporadic: most commonly afflicts people aged 40 to 60 years
- VHL disease: afflicts younger people
- Slight male gender predilection
- Common presenting signs/symptoms related to increased intracranial pressure due to posterior fossa obstructive hydrocephalus
- Vomiting, altered mental status, macrocephaly
- Treatment: surgical resection
- Favorable prognosis: 10-year survival, 85%
- Tumor recurrence in up to 20%
- Leptomeningeal dissemination rare
- Posterior fossa mass
- Supratentorial, retinal HGBLs in patients with VHL disease
- Cyst with enhancing mural nodule common morphology but sometimes solid without cyst
- May have surrounding edema, particularly in the setting of hemorrhage
- Posterior fossa mass
- Hypodense cyst
- Isodense to hyperdense solid nodule
- Rare hyperdense hemorrhage, often with surrounding hypodense cerebellar edema
- Solid nodular component will enhance on contrast-enhanced CT
- T1WI: isointense nodule, ±flow voids
- T2WI: hyperintense cyst and nodule, possible surrounding hyperintense edema
- FLAIR: hyperintense cyst and nodule, possible surrounding hyperintense edema
- T2*GRE: hypointense signal if hemorrhage is present
- T1WI+C: avidly enhancing nodule; nonenhancing cyst, usually without cyst wall enhancement
- DWI: possible DWI-bright, ADC-dark diffusion restriction in cystic component
- MRS: elevated Cho, decreased NAA, ±lactate/lipid peaks
- MRI without and with intravenous contrast; screen patients with VHL disease, including both brain and spine, beginning in early childhood
For more information, please see the corresponding chapter in Radiopaedia. For additional information on spinal HGBLs, please refer to the Hemangioblastoma chapter within the spinal disorders subvolume.
Contributors: Rachel Seltman, MD, and Jacob A. Eitel, MD
Conway JE, Chou D, Clatterbuck RE, et al. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 2001;48:55–62, discussion 62–63.
Innus C, Patterson J. Hemangioblastoma without von Hippel-Lindau disease. JAAPA 2007;20:28–32.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier; 2016.
Wanebo JE, Lonser RR, Glenn GM, et al. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003;98:82–94.
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