{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T06:47:15Z","timestamp":1776494835026,"version":"3.51.2"},"reference-count":44,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,1,24]],"date-time":"2023-01-24T00:00:00Z","timestamp":1674518400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81770781, 81472594"],"award-info":[{"award-number":["81770781, 81472594"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004735","name":"Natural Science Foundation of Hunan Province, China","doi-asserted-by":"publisher","award":["2019JJ50978"],"award-info":[{"award-number":["2019JJ50978"]}],"id":[{"id":"10.13039\/501100004735","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Surg."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Supratentorial hemangioblastoma is an extremely rare neoplasm. The aim of this study is to delineate the clinical features among cystic and solid supratentorial hemangioblastoma patients and evaluate the risk factors for progression-free survival (PFS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a literature search in PubMed for histopathologically identified supratentorial hemangioblastoma between 1947 and 2021 and extracted and collected the clinical features of patients treated at our own institute. The rate of PFS was determined using Kaplan\u2013Meier analysis. Differences in categorical factors, such as the location of tumor and diagnosis of von Hippel\u2013Lindau disease, were analyzed using the Pearson <jats:italic>\u03c7<\/jats:italic><jats:sup>2<\/jats:sup> test. A Cox regression analysis was performed to evaluate the association between various variates and survival outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 237 cases of supratentorial hemangioblastoma were identified from 169 studies. A survival analysis found that patients with cystic tumors had a significantly better prognosis than those with solid tumors (log-rank, <jats:italic>p<\/jats:italic>\u2009=\u20090.0122). Cox regression analysis suggested that cystic hemangioblastoma (hazard ratio (HR): 0.186, 95% CI: 0.043\u20130.803, <jats:italic>p<\/jats:italic>\u2009&amp;lt;\u20090.05) and gross total resection (GTR) (HR: 0.126, 95% CI: 0.049\u20130.323, <jats:italic>p<\/jats:italic>\u2009&amp;lt;\u20090.001) were significant predictors of longer survival (PFS) for supratentorial hemangioblastoma. Following an analysis of 13 supratentorial hemangioblastoma cases from our institute, we validated that cystic tumor had improved prognosis than solid tumor (log-rank, <jats:italic>p<\/jats:italic>\u2009=\u20090.0096) and GTR was superior to subtotal resection (log-rank, <jats:italic>p<\/jats:italic>\u2009=\u20090.0029).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Cystic hemangioblastoma vs. solid hemangioblastoma may be two tumoral statuses with different clinical features, and a specific treatment strategy should be considered.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fsurg.2022.1092140","type":"journal-article","created":{"date-parts":[[2023,1,24]],"date-time":"2023-01-24T05:35:44Z","timestamp":1674538544000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Clinical characteristics, surgical management, and prognostic factors for supratentorial hemangioblastoma: A retrospective study"],"prefix":"10.3389","volume":"9","author":[{"given":"Long","family":"Chen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zujian","family":"Xiong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yian","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yanwen","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuanyang","family":"Xie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yi","family":"Xiong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siyi","family":"Wanggou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xuejun","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,1,24]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1080\/00207454.2017.1385613","article-title":"Supratentorial hemangioblastomas in von Hippel\u2013Lindau wild-type patients\u2014case series and literature review","volume":"128","author":"Rocha","year":"2018","journal-title":"Int J Neurosci"},{"key":"B2","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1111\/j.1365-2613.2007.00535.x","article-title":"Central nervous system capillary haemangioblastoma: the pathologist\u2019s viewpoint","volume":"88","author":"Hussein","year":"2007","journal-title":"Int J Exp Pathol"},{"key":"B3","first-page":"7051","article-title":"Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel\u2013Lindau disease","volume":"63","author":"Vortmeyer","year":"2003","journal-title":"Cancer Res"},{"key":"B4","doi-asserted-by":"publisher","first-page":"514","DOI":"10.1002\/path.2435","article-title":"Protracted haemangioblastic proliferation and differentiation in von Hippel\u2013Lindau disease","volume":"216","author":"Shively","year":"2008","journal-title":"J Pathol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"4102","DOI":"10.1038\/srep04102","article-title":"Tumor derived vasculogenesis in von Hippel\u2013Lindau disease-associated tumors","volume":"4","author":"Zhuang","year":"2014","journal-title":"Sci Rep"},{"key":"B6","doi-asserted-by":"publisher","first-page":"210","DOI":"10.3171\/JNS\/2008\/108\/2\/0210","article-title":"Surgical management of cerebellar hemangioblastomas in patients with von Hippel\u2013Lindau disease","volume":"108","author":"Jagannathan","year":"2008","journal-title":"J Neurosurg"},{"key":"B7","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1227\/01.NEU.0000374846.86409.A7","article-title":"Natural history of supratentorial hemangioblastomas in von Hippel\u2013Lindau disease","volume":"67","author":"Peyre","year":"2010","journal-title":"Neurosurgery"},{"key":"B8","doi-asserted-by":"publisher","first-page":"548","DOI":"10.23736\/S0390-5616.17.04153-4","article-title":"von Hippel\u2013Lindau disease: when neurosurgery meets nephrology, ophthalmology and genetics","volume":"63","author":"Signorelli","year":"2019","journal-title":"J Neurosurg Sci"},{"key":"B9","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1002\/ajmg.a.33139","article-title":"Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service","author":"Evans","year":"2010","journal-title":"Am J Med Genet A"},{"key":"B10","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1002\/ana.10807","article-title":"Somatic mutations in VHL germline deletion kindred correlate with mild phenotype","volume":"55","author":"Wait","year":"2004","journal-title":"Ann Neurol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1136\/jmedgenet-2017-104995","article-title":"Risk factors for survival in patients with von Hippel\u2013Lindau disease","volume":"55","author":"Wang","year":"2018","journal-title":"J Med Genet"},{"key":"B12","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1212\/WNL.41.1.41","article-title":"Central nervous system involvement in von Hippel\u2013Lindau disease","volume":"41","author":"Filling-Katz","year":"1991","journal-title":"Neurology"},{"key":"B13","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1001\/jama.275.11.839","article-title":"von Hippel\u2013Lindau disease and pheochromocytoma","volume":"275","author":"Walther","year":"1996","journal-title":"JAMA"},{"key":"B14","doi-asserted-by":"publisher","first-page":"898","DOI":"10.1136\/jnnp.55.10.898","article-title":"Central nervous system lesions in von Hippel\u2013Lindau syndrome","volume":"55","author":"Neumann","year":"1992","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B15","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.1016\/S0140-6736(03)13643-4","article-title":"von Hippel\u2013Lindau disease","volume":"361","author":"Lonser","year":"2003","journal-title":"Lancet"},{"key":"B16","doi-asserted-by":"publisher","first-page":"1115","DOI":"10.3171\/2018.6.JNS181132","article-title":"Surgical management and prognostic factors for primary intracranial myxoma: a single-institute experience with a systematic review","volume":"131","author":"Weng","year":"2018","journal-title":"J Neurosurg"},{"key":"B17","doi-asserted-by":"publisher","first-page":"545","DOI":"10.3171\/jns.1990.73.4.0545","article-title":"Growth rate of incidental meningiomas","volume":"73","author":"Firsching","year":"1990","journal-title":"J Neurosurg"},{"key":"B18","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1007\/s11060-019-03213-z","article-title":"Growth rate and fate of untreated hemangioblastomas: clinical assessment of the experience of a single institution","volume":"144","author":"Byun","year":"2019","journal-title":"J Neurooncol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"1055","DOI":"10.3171\/2014.1.JNS131431","article-title":"Prospective natural history study of central nervous system hemangioblastomas in von Hippel\u2013Lindau disease","volume":"120","author":"Lonser","year":"2014","journal-title":"J Neurosurg"},{"key":"B20","doi-asserted-by":"publisher","first-page":"82","DOI":"10.3171\/jns.2003.98.1.0082","article-title":"The natural history of hemangioblastomas of the central nervous system in patients with von Hippel\u2013Lindau disease","volume":"98","author":"Wanebo","year":"2003","journal-title":"J Neurosurg"},{"key":"B21","doi-asserted-by":"publisher","first-page":"1097","DOI":"10.1093\/neuonc\/nos133","article-title":"Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel\u2013Lindau disease","volume":"14","author":"Mills","year":"2012","journal-title":"Neuro Oncol"},{"key":"B22","doi-asserted-by":"publisher","first-page":"971","DOI":"10.3171\/2015.4.JNS1533","article-title":"Biological and clinical impact of hemangioblastoma-associated peritumoral cysts in von Hippel\u2013Lindau disease","volume":"124","author":"Huntoon","year":"2016","journal-title":"J Neurosurg"},{"key":"B23","doi-asserted-by":"publisher","first-page":"639","DOI":"10.1007\/s002340000363","article-title":"On the pathogenesis of brain tumour cysts: a volumetric study of tumour, oedema and cyst","volume":"42","author":"Lohle","year":"2000","journal-title":"Neuroradiology"},{"key":"B24","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1172\/JCI116165","article-title":"Expression of the vascular permeability factor\/vascular endothelial growth factor gene in central nervous system neoplasms","volume":"91","author":"Berkman","year":"1993","journal-title":"J Clin Invest"},{"key":"B25","doi-asserted-by":"publisher","first-page":"1334","DOI":"10.1001\/jama.300.11.1334","article-title":"Neurologic manifestations of von Hippel\u2013Lindau disease","volume":"300","author":"Butman","year":"2008","journal-title":"JAMA"},{"key":"B26","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/B978-0-444-62702-5.00010-X","article-title":"von Hippel\u2013Lindau disease","volume":"132","author":"Chittiboina","year":"2015","journal-title":"Handb Clin Neurol"},{"key":"B27","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1016\/j.jocn.2004.02.025","article-title":"Suprasellar haemangioblastoma. Report of two cases and review of the literature","volume":"12","author":"Peker","year":"2005","journal-title":"J Clin Neurosci"},{"key":"B28","first-page":"7553","article-title":"Suprasellar hemangioblastoma without von Hippel\u2013Lindau disease: a case report and literature review","volume":"8","author":"Li","year":"2015","journal-title":"Int J Clin Exp Pathol"},{"key":"B29","doi-asserted-by":"publisher","first-page":"e661","DOI":"10.1055\/s-0042-1757618","article-title":"Pituitary stalk hemangioblastoma: complete resection through orbitozygomatic approach with extradural anterior clinoidectomy","volume":"83","author":"Messina","year":"2022","journal-title":"J Neurol Surg B Skull Base"},{"key":"B30","doi-asserted-by":"publisher","first-page":"1165","DOI":"10.1016\/0360-3016(90)90454-R","article-title":"Radiotherapeutic considerations in the treatment of hemangioblastomas of the central nervous system","volume":"18","author":"Smalley","year":"1990","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"B31","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1016\/j.ijrobp.2007.05.025","article-title":"Role of fractionated external beam radiotherapy in hemangioblastoma of the central nervous system","volume":"69","author":"Koh","year":"2007","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"B32","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1093\/neuonc\/not201","article-title":"The long-term outcomes of radiosurgery for intracranial hemangioblastomas","volume":"16","author":"Hanakita","year":"2014","journal-title":"Neuro Oncol"},{"key":"B33","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1093\/neuonc\/nop018","article-title":"Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel\u2013Lindau disease","volume":"12","author":"Asthagiri","year":"2010","journal-title":"Neuro Oncol"},{"key":"B34","doi-asserted-by":"publisher","first-page":"1469","DOI":"10.3171\/2014.10.JNS131602","article-title":"Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study","volume":"122","author":"Kano","year":"2015","journal-title":"J Neurosurg"},{"key":"B35","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1016\/j.wneu.2010.09.032","article-title":"Gamma knife radiosurgery for intracranial hemangioblastomas\u2014outcome at 3 years","volume":"75","author":"Sayer","year":"2011","journal-title":"World Neurosurg"},{"key":"B36","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1016\/j.jocn.2016.03.008","article-title":"Gamma knife radiosurgery for intracranial hemangioblastoma","volume":"31","author":"Silva","year":"2016","journal-title":"J Clin Neurosci"},{"key":"B37","doi-asserted-by":"publisher","first-page":"471","DOI":"10.1007\/s11060-019-03118-x","article-title":"Gamma knife stereotactic radiosurgery favorably changes the clinical course of hemangioblastoma growth in von Hippel\u2013Lindau and sporadic patients","volume":"142","author":"Liebenow","year":"2019","journal-title":"J Neurooncol"},{"key":"B38","doi-asserted-by":"publisher","first-page":"620","DOI":"10.1001\/jamaneurol.2017.4469","article-title":"Review of the neurological implications of von Hippel\u2013Lindau disease","volume":"75","author":"Dornbos","year":"2018","journal-title":"JAMA Neurol"},{"key":"B39","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1227\/01.NEU.0000348015.51685.D2","article-title":"Stereotactic radiosurgical treatment of cranial and spinal hemangioblastomas","volume":"65","author":"Moss","year":"2009","journal-title":"Neurosurgery"},{"key":"B40","doi-asserted-by":"publisher","first-page":"105912","DOI":"10.1016\/j.clineuro.2020.105912","article-title":"Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel\u2013Lindau disease: a systematic review and meta-analysis","volume":"195","author":"Qiu","year":"2020","journal-title":"Clin Neurol Neurosurg"},{"key":"B41","doi-asserted-by":"publisher","first-page":"1030","DOI":"10.1093\/neuonc\/nor085","article-title":"Infratentorial craniospinal irradiation for von Hippel\u2013Lindau: a retrospective study supporting a new treatment for patients with CNS hemangioblastomas","volume":"13","author":"Simone","year":"2011","journal-title":"Neuro Oncol"},{"key":"B42","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1016\/j.critrevonc.2012.10.001","article-title":"Mechanisms, indications and results of salvage systemic therapy for sporadic and von Hippel\u2013Lindau related hemangioblastomas of the central nervous system","volume":"86","author":"Capitanio","year":"2013","journal-title":"Crit Rev Oncol Hematol"},{"key":"B43","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/j.celrep.2012.12.007","article-title":"Proteostasis modulators prolong missense VHL protein activity and halt tumor progression","volume":"3","author":"Yang","year":"2013","journal-title":"Cell Rep"},{"key":"B44","doi-asserted-by":"publisher","first-page":"2520","DOI":"10.1038\/emboj.2011.162","article-title":"Cellular stress response pathways and ageing: intricate molecular relationships","volume":"30","author":"Kourtis","year":"2011","journal-title":"EMBO J"}],"container-title":["Frontiers in Surgery"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fsurg.2022.1092140\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,1,24]],"date-time":"2023-01-24T05:35:51Z","timestamp":1674538551000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fsurg.2022.1092140\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,24]]},"references-count":44,"alternative-id":["10.3389\/fsurg.2022.1092140"],"URL":"https:\/\/doi.org\/10.3389\/fsurg.2022.1092140","relation":{},"ISSN":["2296-875X"],"issn-type":[{"value":"2296-875X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,24]]},"article-number":"1092140"}}