Intracranial hemangioblastoma
Definition
Intracranial hemangioblastoma is a World Health Organization (WHO) grade I tumor of the CNS composed of capillary-sized blood vessels and stromal cells with variable lipid content. It can occur sporadically or in association with von Hippel-Lindau (VHL) disease.
🧠 Key Features
Location: Most often posterior fossa (especially cerebellum).
Histology: Highly vascular with stromal cells, often with foamy cytoplasm.
Clinical presentation: Depends on size and location; may include headache, ataxia, visual disturbances, or signs of increased intracranial pressure due to cyst formation or mass effect.
Radiology: Cystic lesion with an enhancing mural nodule on contrast MRI is characteristic.
Management: Surgical resection is the treatment of choice; stereotactic radiosurgery is an option for select cases.
Prognosis: Excellent for sporadic lesions after complete resection; more complex in VHL-associated cases due to multiplicity.