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Central Nervous System Hemangioblastoma

MedGen UID:
272702
•Concept ID:
C1333955
•
Neoplastic Process
Synonyms: Central Nervous System Capillary Hemangioblastoma; CNS Hemangioblastoma; Hemangioblastoma of Central Nervous System; Hemangioblastoma of CNS; Hemangioblastoma of the CNS

Definition

A rare, slow-growing, WHO grade I tumor of uncertain histogenesis that arises from the central nervous system. It is composed of stromal cells and abundant capillaries. It can occur sporadically or as part of von Hippel- Lindau syndrome. [from NCI]

Professional guidelines

PubMed

Management and outcomes of pregnant patients with central nervous system hemangioblastoma.
Ma XJ, Zhang GB, Guo TX, Li CB, Chen YJ, Zhang LW, Wu Z, Zhang JT
J Clin Neurosci 2018 Nov;57:126-130. Epub 2018 Sep 24 doi: 10.1016/j.jocn.2018.08.009. PMID: 30262382
Genotype and phenotype correlation in von Hippel-Lindau disease based on alteration of the HIF-α binding site in VHL protein.
Liu SJ, Wang JY, Peng SH, Li T, Ning XH, Hong BA, Liu JY, Wu PJ, Zhou BW, Zhou JC, Qi NN, Peng X, Zhang JF, Ma KF, Cai L, Gong K
Genet Med 2018 Oct;20(10):1266-1273. Epub 2018 Mar 29 doi: 10.1038/gim.2017.261. PMID: 29595810
Genotype-phenotype correlations in families with deletions in the von Hippel-Lindau (VHL) gene.
Hes F, Zewald R, Peeters T, Sijmons R, Links T, Verheij J, Matthijs G, Leguis E, Mortier G, van der Torren K, Rosman M, Lips C, Pearson P, van der Luijt R
Hum Genet 2000 Apr;106(4):425-31. doi: 10.1007/s004390000265. PMID: 10830910

Recent clinical studies

Etiology

Epidemiology and economic burden of Von Hippel-Lindau Disease-associated central nervous system hemangioblastomas and pancreatic neuroendocrine tumors in the United States.
Jonasch E, Song Y, Freimark J, Berman R, Nguyen H, Signorovitch J, Sundaram M
Orphanet J Rare Dis 2024 Feb 16;19(1):73. doi: 10.1186/s13023-024-03060-w. PMID: 38365728Free PMC Article
VHL: Trends and Insight into a Multi-Modality, Interdisciplinary Approach for Management of Central Nervous System Hemangioblastoma.
Matloob SA, Paraskevopoulos D, O'Toole SM, Drake W, Plowman N, Foroglou N
Acta Neurochir Suppl 2023;135:81-88. doi: 10.1007/978-3-031-36084-8_14. PMID: 38153453
Belzutifan in adults with VHL-associated central nervous system hemangioblastoma: a single-center experience.
Neth BJ, Webb MJ, White J, Uhm JH, Pichurin PN, Sener U
J Neurooncol 2023 Aug;164(1):239-247. Epub 2023 Jul 14 doi: 10.1007/s11060-023-04395-3. PMID: 37450072
Impact of Pancreatic Neuroendocrine Tumor on Mortality in Patients With von Hippel-Lindau Disease.
Arnon L, Halperin R, Tirosh A
Endocr Pract 2021 Oct;27(10):1040-1045. Epub 2021 Mar 15 doi: 10.1016/j.eprac.2021.03.005. PMID: 33737209
Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease.
Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH
J Neurosurg 2014 May;120(5):1055-62. Epub 2014 Feb 28 doi: 10.3171/2014.1.JNS131431. PMID: 24579662Free PMC Article

Diagnosis

Disseminated central nervous system hemangioblastoma in a patient with no clinical or genetic evidence of von Hippel-Lindau disease-a case report and literature review.
Bains SJ, Niehusmann PF, Meling TR, Saxhaug C, Züchner M, Brandal P
Acta Neurochir (Wien) 2019 Feb;161(2):343-349. Epub 2019 Jan 17 doi: 10.1007/s00701-019-03800-z. PMID: 30652202
Management and outcomes of pregnant patients with central nervous system hemangioblastoma.
Ma XJ, Zhang GB, Guo TX, Li CB, Chen YJ, Zhang LW, Wu Z, Zhang JT
J Clin Neurosci 2018 Nov;57:126-130. Epub 2018 Sep 24 doi: 10.1016/j.jocn.2018.08.009. PMID: 30262382
Multifocal central nervous system hemangioblastoma: a case report and review of the literature.
Chu LZ, Guan ZZ, Liu J, Yang H, Qi XL, Dong MG, Chen YM, Xiang YN, Li Y
Genet Mol Res 2014 Sep 29;13(3):7904-11. doi: 10.4238/2014.September.29.3. PMID: 25299105
Peripheral hemangioblastoma: clinicopathologic characterization in a series of 22 cases.
Doyle LA, Fletcher CD
Am J Surg Pathol 2014 Jan;38(1):119-27. doi: 10.1097/PAS.0b013e3182a266c1. PMID: 24145646
Soft-tissue hemangioblastoma of the retroperitoneum: a case study and review of the literature.
Yoshida A, Oda R, Shibahara J, Fukayama M, Tsuda H
Appl Immunohistochem Mol Morphol 2010 Oct;18(5):479-82. doi: 10.1097/PAI.0b013e3181daad26. PMID: 20436343

Therapy

Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel-Lindau disease: A systematic review and meta-analysis.
Qiu J, Cai D, Yang F, Zhou J, Gong Y, Cai L, Gong K
Clin Neurol Neurosurg 2020 Aug;195:105912. Epub 2020 May 15 doi: 10.1016/j.clineuro.2020.105912. PMID: 32474257
Effect of clarithromycin in von Hippel-Lindau syndrome: a case report.
Ma X, Jing Y, Liu Y, Yu L
J Int Med Res 2019 Feb;47(2):973-981. Epub 2018 Sep 4 doi: 10.1177/0300060518792368. PMID: 30178691Free PMC Article
Management and outcomes of pregnant patients with central nervous system hemangioblastoma.
Ma XJ, Zhang GB, Guo TX, Li CB, Chen YJ, Zhang LW, Wu Z, Zhang JT
J Clin Neurosci 2018 Nov;57:126-130. Epub 2018 Sep 24 doi: 10.1016/j.jocn.2018.08.009. PMID: 30262382
Stereotactic radiosurgery for central nervous system hemangioblastoma: systematic review and meta-analysis.
Pan J, Jabarkheel R, Huang Y, Ho A, Chang SD
J Neurooncol 2018 Mar;137(1):11-22. Epub 2017 Dec 4 doi: 10.1007/s11060-017-2697-0. PMID: 29204841
Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease.
Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH
J Neurosurg 2014 May;120(5):1055-62. Epub 2014 Feb 28 doi: 10.3171/2014.1.JNS131431. PMID: 24579662Free PMC Article

Prognosis

Epidemiology and economic burden of Von Hippel-Lindau Disease-associated central nervous system hemangioblastomas and pancreatic neuroendocrine tumors in the United States.
Jonasch E, Song Y, Freimark J, Berman R, Nguyen H, Signorovitch J, Sundaram M
Orphanet J Rare Dis 2024 Feb 16;19(1):73. doi: 10.1186/s13023-024-03060-w. PMID: 38365728Free PMC Article
Disseminated central nervous system hemangioblastoma in a patient with no clinical or genetic evidence of von Hippel-Lindau disease-a case report and literature review.
Bains SJ, Niehusmann PF, Meling TR, Saxhaug C, Züchner M, Brandal P
Acta Neurochir (Wien) 2019 Feb;161(2):343-349. Epub 2019 Jan 17 doi: 10.1007/s00701-019-03800-z. PMID: 30652202
Management and outcomes of pregnant patients with central nervous system hemangioblastoma.
Ma XJ, Zhang GB, Guo TX, Li CB, Chen YJ, Zhang LW, Wu Z, Zhang JT
J Clin Neurosci 2018 Nov;57:126-130. Epub 2018 Sep 24 doi: 10.1016/j.jocn.2018.08.009. PMID: 30262382
Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease.
Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH
J Neurosurg 2014 May;120(5):1055-62. Epub 2014 Feb 28 doi: 10.3171/2014.1.JNS131431. PMID: 24579662Free PMC Article
Peripheral hemangioblastoma: clinicopathologic characterization in a series of 22 cases.
Doyle LA, Fletcher CD
Am J Surg Pathol 2014 Jan;38(1):119-27. doi: 10.1097/PAS.0b013e3182a266c1. PMID: 24145646

Clinical prediction guides

Management and outcomes of pregnant patients with central nervous system hemangioblastoma.
Ma XJ, Zhang GB, Guo TX, Li CB, Chen YJ, Zhang LW, Wu Z, Zhang JT
J Clin Neurosci 2018 Nov;57:126-130. Epub 2018 Sep 24 doi: 10.1016/j.jocn.2018.08.009. PMID: 30262382
Stereotactic radiosurgery for central nervous system hemangioblastoma: systematic review and meta-analysis.
Pan J, Jabarkheel R, Huang Y, Ho A, Chang SD
J Neurooncol 2018 Mar;137(1):11-22. Epub 2017 Dec 4 doi: 10.1007/s11060-017-2697-0. PMID: 29204841
Sporadic hemangioblastomas are characterized by cryptic VHL inactivation.
Shankar GM, Taylor-Weiner A, Lelic N, Jones RT, Kim JC, Francis JM, Abedalthagafi M, Borges LF, Coumans JV, Curry WT, Nahed BV, Shin JH, Paek SH, Park SH, Stewart C, Lawrence MS, Cibulskis K, Thorner AR, Van Hummelen P, Stemmer-Rachamimov AO, Batchelor TT, Carter SL, Hoang MP, Santagata S, Louis DN, Barker FG, Meyerson M, Getz G, Brastianos PK, Cahill DP
Acta Neuropathol Commun 2014 Dec 24;2:167. doi: 10.1186/s40478-014-0167-x. PMID: 25589003Free PMC Article
Multifocal central nervous system hemangioblastoma: a case report and review of the literature.
Chu LZ, Guan ZZ, Liu J, Yang H, Qi XL, Dong MG, Chen YM, Xiang YN, Li Y
Genet Mol Res 2014 Sep 29;13(3):7904-11. doi: 10.4238/2014.September.29.3. PMID: 25299105
Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease.
Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH
J Neurosurg 2014 May;120(5):1055-62. Epub 2014 Feb 28 doi: 10.3171/2014.1.JNS131431. PMID: 24579662Free PMC Article

Recent systematic reviews

Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel-Lindau disease: A systematic review and meta-analysis.
Qiu J, Cai D, Yang F, Zhou J, Gong Y, Cai L, Gong K
Clin Neurol Neurosurg 2020 Aug;195:105912. Epub 2020 May 15 doi: 10.1016/j.clineuro.2020.105912. PMID: 32474257
Stereotactic radiosurgery for central nervous system hemangioblastoma: systematic review and meta-analysis.
Pan J, Jabarkheel R, Huang Y, Ho A, Chang SD
J Neurooncol 2018 Mar;137(1):11-22. Epub 2017 Dec 4 doi: 10.1007/s11060-017-2697-0. PMID: 29204841

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