Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2014 Aug 5;1(4):46-55.
doi: 10.15586/jkcvhl.2014.12. eCollection 2014.

Pathological and Clinical Features and Management of Central Nervous System Hemangioblastomas in von Hippel-Lindau Disease

Hiroshi Kanno  1 , Natsuki Kobayashi  1 , Satoshi Nakanowatari  1
Affiliations

Pathological and Clinical Features and Management of Central Nervous System Hemangioblastomas in von Hippel-Lindau Disease

Hiroshi Kanno et al. J Kidney Cancer VHL. .

Abstract

Central nervous system (CNS) hemangioblastoma is the most common manifestation of von Hippel-Lindau (VHL) disease. It is found in 70-80% of VHL patients. Hemangioblastoma is a rare form of benign vascular tumor of the CNS, accounting for 2.0% of CNS tumors. It can occur sporadically or as a familial syndrome. CNS hemangioblastomas are typically located in the posterior fossa and the spinal cord. VHL patients usually develop a CNS hemangioblastoma at an early age. Therefore, they require a special routine for diagnosis, treatment and follow-up. The surgical management of symptomatic tumors depend on many factors such as symptom, location, multiplicity, and progression of the tumor. The management of asymptomatic tumors in VHL patients are controversial since CNS hemangioblastomas grow with intermittent quiescent and rapid-growth phases. Preoperative embolization of large solid hemangioblastomas prevents perioperative hemorrhage but is not necessary in every case. Radiotherapy should be reserved for inoperable tumors. Because of complexities of VHL, a better understanding of the pathological and clinical features of hemangioblastoma in VHL is essential for its proper management.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest: None.

Figures

Figure 1.
Figure 1.
The interaction of VHL protein with HIF and other proteins including elongin B, elongin C and CUL2. A mutated VHL stabilizes HIF and leads to the up-regulation of many pro-angiogenic factors including GLUT-I, VEGF, PDGF-β, erythropoietin and TGF-α. A wild type VHL degrades HIF through ubiquitin-mediated pathway.
Figure 2.
Figure 2.
Contrast-enhanced T1 weighted MRI of CNS HBs in VHL. Left, multi-cerebellar HBs; right, lumbar spinal cord HB with syrinx.
Figure 3.
Figure 3.
Clinical management of hemangioblastomas.
See this image and copyright information in PMC

References

    1. Huntoon K, Lonser RR. Nervous system manifestations of von Hippel-Lindau disease. J Transl Med Epidemiol. 2014;2(1):1015.
    1. Vortmeyer AO, Frank S, Jeong SY, Yuan K, Ikejiri B, Lee YS, Bhowmick D, Lonser RR, Smith R, Rodgers G, Oldfield EH, Zhuang Z. Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel-Lindau disease. Cancer Res. 2003;63(21):7051–7055. Pubmed. - PubMed
    1. Glasker S, Li J, Xia JB, Okamoto H, Zeng W, Lonser RR, Zhuang Z, Oldfield EH, Vortmeyer AO. Hemangioblastomas share protein expression with embryonal hemangioblast progenitor cell. Cancer Res. 2006;66(8):4167–4172. http://dx.doi.org/10.1158/0008-5472.CAN-05-3505. - DOI - PubMed
    1. Hasselblatt M, Jeibmann A, Gerss J, Behrens C, Rama B, Wassmann H, Paulus W. Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. Neuropathol Appl Neurobiol. 2005;31(6):618–622. http://dx.doi.org/10.1111/j.1365-2990.2005.00669.x. - DOI - PubMed
    1. Böhling T1, Mäenpää A, Timonen T, Vantunen L, Paetau A, Haltia M. Different expression of adhesion molecules on stromal cells and endothelial cells of capillary hemangioblastoma. Acta Neuropathol. 1996;92(5):461–466. http://dx.doi.org/10.1007/s004010050547. - DOI - PubMed

LinkOut - more resources