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

Abstract

Background: 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.

Methods: 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.

Results: Based upon our literature search, we established guidelines for screening and follow-up in both sporadic and VHL associated hemangioblastoma patients.

Conclusions: 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.

Keywords: Diagnosis; Von Hippel-Lindau disease; follow-up; hemangioblastoma; work-up.

Figure 1

Perioperative image of a hemangioblastoma. Note the high degree of vascularity

Figure 2

MR imaging of a cerebellar hemangioblastoma. Note the characteristic contrast enhancing nodule associated with a peritumoral cyst in the cerebellum. These lesions appear as a low signal on T1-weighted images (a) and as a high signal on T2-weighted sequences (b)

Figure 3

MR imaging of a spinal hemangioblastoma. Note the homogeneously contrast enhancing lesion in the spinal cord with the associated syrinx

Figure 4

Histopathological examination of a hemangioblastoma. Note the extensive vascular network with neoplastic stromal cells. The neoplastic stromal cells have abundant cytoplasm with lipid vacuoles resulting in a typical clear cell morphology”