hemangioblastoma
Pronunciation: hee-MAN-jee-oh-blas-TOH-mah
A benign, highly vascularized tumor of the central nervous system, often associated with von Hippel-Lindau disease.
Full Definition
A hemangioblastoma is a benign, WHO grade I tumor characterized by abundant capillary networks and distinctive stromal cells. These tumors most commonly occur in the cerebellum and spinal cord, presenting as cystic masses with mural nodules. Hemangioblastomas may occur sporadically or as part of von Hippel-Lindau (VHL) disease, an inherited tumor syndrome. Histologically, they show a rich vascular network with intervening stromal cells that are often vacuolated and may contain lipid droplets. The stromal cells are positive for inhibin-α and may show nuclear expression of hypoxia-inducible factors.
Usage
Usage note: Strong association with VHL disease; inhibin-α staining is characteristic and diagnostically useful.
In Context
- "The cerebellar hemangioblastoma showed the characteristic highly vascular architecture with intervening stromal cells." — Neuropathology report
- "Given the patient's family history, genetic counseling for von Hippel-Lindau disease was recommended following hemangioblastoma diagnosis." — Clinical consultation note