What the neurosurgeon should know about hemangioblastoma, both sporadic and in Von Hippel-Lindau disease: A literature review.

[von hippel-lindau disease]

Hemangioblastomas are associated with Von Hippel-Lindau disease (VHLD ) in 10 -40 % of cases . Based upon a literature review we state the core features the neurosurgeon should be aware of .We performed a selective literature (Cochrane and Medline ) search for hemangioblastoma , both sporadic and VHL associated . We reviewed general characteristics (epidemiology , symptomatology , diagnosis , and management ) and focused on follow-up as well as screening modalities for sporadic and VHL associated lesions .Based upon our literature search , we established guidelines for screening and follow-up in both sporadic and VHL associated hemangioblastoma patients .Screening for retinal angiomas , abdominal masses , and pheochromocytomas as well as genetic analysis is recommended for every patient with a newly diagnosed hemangioblastoma . Follow-up is by magnetic resonance imaging (MRI ) of the clinical neuronal region at 6 and at 12 -24 months postoperatively . For VHL -associated hemangioblastomas yearly investigation for craniospinal hemangioblastoma by MRI and yearly screening and follow-up for retinal angiomas is recommended . Annual abdominal ultrasound with triennial computed tomography (CT ) imaging for abdominal masses is postulated . Annual audiometry is to be performed for possible endolymphatic sac tumor , detailed radiographic imaging of the skull base should be performed upon abnormality in auditory testing . Investigations for cystadenomas of the epidydimis and broad ligament only are mandatory on indication . Annual investigation for pheochromocytoma is recommended .