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Most haemangioblastomas are sporadic but up to one quarter are associated with Von Hippel\u2013Lindau disease (VHL). Although a fairly good number of haemangioblastomas were reported, a lack of side\u2010by\u2010side analysis of these reports has resulted in tentative conclusions that merely offer a first glimpse at their clinicopathologic diversity and histogenesis. To remedy this issue, this study presents a literature review concerning these lesions. Medline literature including both relevant monographs and clinicopathological case series. Haemangioblastomas occur either as a part of VHL disease (25\u201330%, inherited mutation of VHL gene on 3p25\u201326 chromosome) or as sporadic tumours (often with somatic mutation of VHL gene). They have diverse clinicopathologic presentations with cerebellar lesions having a better prognosis than their brainstem counterparts. Immunostaining is important for separation of haemangioblastomas from other tumours with similar histology. The rich vascularity of haemangioblastomas is due to overexpression of vascular endothelial growth factors. Moreover, \u2018stromal\u2019 cells represent the neoplastic cells of haemangioblastomas and are capable of forming blood islands with extramedullary haematopoiesis.<\/jats:p>","DOI":"10.1111\/j.1365-2613.2007.00535.x","type":"journal-article","created":{"date-parts":[[2007,5,7]],"date-time":"2007-05-07T12:21:08Z","timestamp":1178540468000},"page":"311-324","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":94,"title":["Central nervous system capillary haemangioblastoma: the pathologist's viewpoint"],"prefix":"10.1111","volume":"88","author":[{"given":"Mahmoud R.","family":"Hussein","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,4,30]]},"reference":[{"key":"e_1_2_13_2_1","first-page":"91","article-title":"[Round table: infratentorial hemangioblastoma]","volume":"31","author":"Acikalin M.F.","year":"1985","journal-title":"Neurochirurgie"},{"key":"e_1_2_13_3_1","doi-asserted-by":"crossref","first-page":"e382","DOI":"10.5858\/2003-127-e382-SHACRA","article-title":"Supratentorial hemangioblastoma: a case report and review of the literature","volume":"127","author":"Acikalin M.F.","year":"2003","journal-title":"Arch. 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