{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:45:19Z","timestamp":1791218719236,"version":"4.1.0"},"reference-count":189,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2013,2,12]],"date-time":"2013-02-12T00:00:00Z","timestamp":1360627200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2013,3]]},"DOI":"10.1007\/s00401-013-1091-z","type":"journal-article","created":{"date-parts":[[2013,2,11]],"date-time":"2013-02-11T11:30:15Z","timestamp":1360582215000},"page":"333-350","source":"Crossref","is-referenced-by-count":50,"title":["Nervous system involvement in von Hippel\u2013Lindau disease: pathology and mechanisms"],"prefix":"10.1007","volume":"125","author":[{"given":"Alexander O.","family":"Vortmeyer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric A.","family":"Falke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sven","family":"Gl\u00e4sker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward H.","family":"Oldfield","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,2,12]]},"reference":[{"issue":"4","key":"1091_CR1","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1093\/jnen\/64.4.341","volume":"64","author":"TW Abel","year":"2005","unstructured":"Abel TW, Baker SJ, Fraser MM et al (2005) Lhermitte\u2013Duclos disease: a report of 31 cases with immunohistochemical analysis of the PTEN\/AKT\/mTOR pathway. J Neuropathol Exp Neurol 64(4):341\u2013349","journal-title":"J Neuropathol Exp Neurol"},{"issue":"3","key":"1091_CR2","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1080\/003130203100023191","volume":"35","author":"A Abou-Hamden","year":"2003","unstructured":"Abou-Hamden A, Koszyca B, Carney PG, Sandhu N, Blumbergs PC (2003) Metastasis of renal cell carcinoma to haemangioblastoma of the spinal cord in von Hippel\u2013Lindau disease: case report and review of the literature. Pathology 35(3):224\u2013227","journal-title":"Pathology"},{"key":"1091_CR3","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1046\/j.1365-2990.2002.00371.x","volume":"28","author":"SA Adams","year":"2002","unstructured":"Adams SA, Hilton DA (2002) Recurrent haemangioblastoma with glial differentiation. Neuropathol Appl Neurobiol 28:142\u2013146","journal-title":"Neuropathol Appl Neurobiol"},{"issue":"8","key":"1091_CR4","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1136\/jcp.39.8.917","volume":"39","author":"SM Andrew","year":"1986","unstructured":"Andrew SM, Gradwell E (1986) Immunoperoxidase labelled antibody staining in differential diagnosis of central nervous system haemangioblastomas and central nervous system metastases of renal carcinomas. J Clin Pathol 39(8):917\u2013919","journal-title":"J Clin Pathol"},{"issue":"1","key":"1091_CR5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1001\/archotol.1977.00780180039001","volume":"103","author":"IK Arenberg","year":"1977","unstructured":"Arenberg IK, Rask-Andersen H, Wilbrand H, Stahle J (1977) The surgical anatomy of the endolymphatic sac. Arch Otolaryngol 103(1):1\u201311","journal-title":"Arch Otolaryngol"},{"issue":"6","key":"1091_CR6","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1046\/j.1365-2265.2003.01914.x","volume":"59","author":"D Astuti","year":"2003","unstructured":"Astuti D, Hart-Holden N, Latif F et al (2003) Genetic analysis of mitochondrial complex II subunits SDHD, SDHB and SDHC in paraganglioma and phaeochromocytoma susceptibility. Clin Endocrinol (Oxf) 59(6):728\u2013733","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"1","key":"1091_CR7","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1086\/321282","volume":"69","author":"D Astuti","year":"2001","unstructured":"Astuti D, Latif F, Dallol A et al (2001) Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet 69(1):49\u201354","journal-title":"Am J Hum Genet"},{"issue":"1","key":"1091_CR8","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1677\/ERC-10-0113","volume":"18","author":"D Astuti","year":"2011","unstructured":"Astuti D, Ricketts CJ, Chowdhury R et al (2011) Mutation analysis of HIF prolyl hydroxylases (PHD\/EGLN) in individuals with features of phaeochromocytoma and renal cell carcinoma susceptibility. Endocr Relat Cancer 18(1):73\u201383","journal-title":"Endocr Relat Cancer"},{"issue":"4","key":"1091_CR9","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/S1470-2045(10)70007-3","volume":"11","author":"JP Bayley","year":"2010","unstructured":"Bayley JP, Kunst HP, Cascon A et al (2010) SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma. Lancet Oncol 11(4):366\u2013372","journal-title":"Lancet Oncol"},{"key":"1091_CR10","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0065-230X(08)60702-2","volume":"72","author":"SB Baylin","year":"1998","unstructured":"Baylin SB, Herman JG, Graff JR, Vertino PM, Issa JP (1998) Alterations in DNA methylation: a fundamental aspect of neoplasia. Adv Cancer Res 72:141\u2013196","journal-title":"Adv Cancer Res"},{"issue":"5454","key":"1091_CR11","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1126\/science.287.5454.848","volume":"287","author":"BE Baysal","year":"2000","unstructured":"Baysal BE, Ferrell RE, Willett-Brozick JE et al (2000) Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Science 287(5454):848\u2013851","journal-title":"Science"},{"key":"1091_CR12","first-page":"271","volume":"134","author":"I Becker","year":"1989","unstructured":"Becker I, Paulus W, Roggendorf W (1989) Histogenesis of stromal cells in cerebellar hemangioblastomas. Am J Pathol 134:271\u2013275","journal-title":"Am J Pathol"},{"issue":"12","key":"1091_CR13","doi-asserted-by":"crossref","first-page":"4568","DOI":"10.1210\/jc.85.12.4568","volume":"85","author":"BU Bender","year":"2000","unstructured":"Bender BU, Gutsche M, Glasker S et al (2000) Differential genetic alterations in von Hippel\u2013Lindau syndrome-associated and sporadic pheochromocytomas. J Clin Endocrinol Metab 85(12):4568\u20134574","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"1091_CR14","doi-asserted-by":"crossref","first-page":"501","DOI":"10.2214\/ajr.155.3.1974734","volume":"155","author":"LA Binkovitz","year":"1990","unstructured":"Binkovitz LA, Johnson CD, Stephens DH (1990) Islet cell tumors in von Hippel\u2013Lindau disease: increased prevalence and relationship to the multiple endocrine neoplasias. AJR Am J Roentgenol 155(3):501\u2013505","journal-title":"AJR Am J Roentgenol"},{"issue":"6","key":"1091_CR15","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1016\/j.surg.2007.09.012","volume":"142","author":"JA Blansfield","year":"2007","unstructured":"Blansfield JA, Choyke L, Morita SY et al (2007) Clinical, genetic and radiographic analysis of 108 patients with von Hippel\u2013Lindau disease (VHL) manifested by pancreatic neuroendocrine neoplasms (PNETs). Surgery 142(6):814\u2013818","journal-title":"Surgery"},{"issue":"11","key":"1091_CR16","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1038\/ejhg.2008.162","volume":"16","author":"GM Blumenthal","year":"2008","unstructured":"Blumenthal GM, Dennis PA (2008) PTEN hamartoma tumor syndromes. Eur J Hum Genet 16(11):1289\u20131300","journal-title":"Eur J Hum Genet"},{"key":"1091_CR17","first-page":"223","volume-title":"Pathology and genetics of tumours of the central nervous system","author":"T Bohling","year":"2000","unstructured":"Bohling T, Plate KH, Haltia M, Alitalo K, Neumann HPH (2000) Von Hippel\u2013Lindau disease and capillary hemangioblastoma. In: Kleihues P, Cawenee WK (eds) Pathology and genetics of tumours of the central nervous system. World Health Organization, Lyon, pp 223\u2013226"},{"issue":"2","key":"1091_CR18","first-page":"146","volume":"16","author":"EL Bold","year":"1995","unstructured":"Bold EL, Wanamaker JR, Hughes GB, Rhee CK, Sebek BA, Kinney SE (1995) Adenomatous lesions of the temporal bone immunohistochemical analysis and theories of histogenesis. Am J Otol 16(2):146\u2013152","journal-title":"Am J Otol"},{"key":"1091_CR19","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1097\/00005072-198309000-00002","volume":"42","author":"JM Bonnin","year":"1983","unstructured":"Bonnin JM, Pena CE, Rubinstein LJ (1983) Mixed capillary hemangioblastoma and glioma. A redefinition of the \u201cangioglioma\u201d. J Neuropathol Exp Neurol 42:504\u2013516","journal-title":"J Neuropathol Exp Neurol"},{"issue":"4","key":"1091_CR20","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1080\/02688699943565","volume":"13","author":"P Bret","year":"1999","unstructured":"Bret P, Streichenberger N, Guyotat J (1999) Metastasis of renal carcinoma to a cerebellar hemangioblastoma in a patient with von Hippel Lindau disease: a case report. Br J Neurosurg 13(4):413\u2013416","journal-title":"Br J Neurosurg"},{"issue":"1","key":"1091_CR21","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.ccr.2004.06.020","volume":"6","author":"J Brugarolas","year":"2004","unstructured":"Brugarolas J, Kaelin WG Jr (2004) Dysregulation of HIF and VEGF is a unifying feature of the familial hamartoma syndromes. Cancer Cell 6(1):7\u201310","journal-title":"Cancer Cell"},{"issue":"1","key":"1091_CR22","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1001\/jama.298.1.41","volume":"298","author":"JA Butman","year":"2007","unstructured":"Butman JA, Kim HJ, Baggenstos M et al (2007) Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel\u2013Lindau disease. JAMA 298(1):41\u201348","journal-title":"JAMA"},{"issue":"11","key":"1091_CR23","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1001\/jama.300.11.1334","volume":"300","author":"JA Butman","year":"2008","unstructured":"Butman JA, Linehan WM, Lonser RR (2008) Neurologic manifestations of von Hippel\u2013Lindau disease. JAMA 300(11):1334\u20131342","journal-title":"JAMA"},{"issue":"4","key":"1091_CR24","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1097\/00005072-196510000-00005","volume":"24","author":"PA Cancilla","year":"1965","unstructured":"Cancilla PA, Zimmerman HM (1965) The fine structure of a cerebellar hemangioblastoma. J Neuropathol Exp Neurol 24(4):621\u2013628","journal-title":"J Neuropathol Exp Neurol"},{"issue":"2","key":"1091_CR25","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1097\/PAS.0b013e3182064d11","volume":"35","author":"EM Carney","year":"2011","unstructured":"Carney EM, Banerjee P, Ellis CL et al (2011) PAX2(\u2212)\/PAX8(\u2212)\/inhibin A(+) immunoprofile in hemangioblastoma: a helpful combination in the differential diagnosis with metastatic clear cell renal cell carcinoma to the central nervous system. Am J Surg Pathol 35(2):262\u2013267","journal-title":"Am J Surg Pathol"},{"issue":"1","key":"1091_CR26","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1002\/path.1174","volume":"198","author":"CC Chan","year":"2002","unstructured":"Chan CC, Koch CA, Kaiser-Kupfer MI et al (2002) Loss of heterozygosity for the NF2 gene in retinal and optic nerve lesions of patients with neurofibromatosis 2. J Pathol 198(1):14\u201320","journal-title":"J Pathol"},{"issue":"5","key":"1091_CR27","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1001\/archopht.117.5.625","volume":"117","author":"CC Chan","year":"1999","unstructured":"Chan CC, Vortmeyer AO, Chew EY et al (1999) VHL gene deletion and enhanced VEGF gene expression detected in the stromal cells of retinal angioma. Arch Ophthalmol 117(5):625\u2013630","journal-title":"Arch Ophthalmol"},{"issue":"3","key":"1091_CR28","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1016\/S0002-9440(10)65641-6","volume":"153","author":"AK Charles","year":"1998","unstructured":"Charles AK, Brown KW, Berry PJ (1998) Microdissecting the genetic events in nephrogenic rests and Wilms\u2019 tumor development. Am J Pathol 153(3):991\u20131000","journal-title":"Am J Pathol"},{"issue":"4","key":"1091_CR29","doi-asserted-by":"crossref","first-page":"1834","DOI":"10.1002\/1097-0142(197810)42:4<1834::AID-CNCR2820420423>3.0.CO;2-Z","volume":"42","author":"AP Chaudhry","year":"1978","unstructured":"Chaudhry AP, Montes M, Cohn GA (1978) Ultrastructure of cerebellar hemangioblastoma. Cancer 42(4):1834\u20131850","journal-title":"Cancer"},{"issue":"3","key":"1091_CR30","doi-asserted-by":"crossref","first-page":"480","DOI":"10.3171\/jns.2004.100.3.0480","volume":"100","author":"D Choo","year":"2004","unstructured":"Choo D, Shotland L, Mastroianni M et al (2004) Endolymphatic sac tumors in von Hippel\u2013Lindau disease. J Neurosurg 100(3):480\u2013487","journal-title":"J Neurosurg"},{"issue":"3","key":"1091_CR31","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1148\/radiology.194.3.7862955","volume":"194","author":"PL Choyke","year":"1995","unstructured":"Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B (1995) von Hippel\u2013Lindau disease: genetic, clinical, and imaging features. Radiology 194(3):629\u2013642","journal-title":"Radiology"},{"issue":"33","key":"1091_CR32","doi-asserted-by":"crossref","first-page":"25733","DOI":"10.1074\/jbc.M002740200","volume":"275","author":"ME Cockman","year":"2000","unstructured":"Cockman ME, Masson N, Mole DR et al (2000) Hypoxia inducible factor-alpha binding and ubiquitylation by the von Hippel\u2013Lindau tumor suppressor protein. J Biol Chem 275(33):25733\u201325741","journal-title":"J Biol Chem"},{"key":"1091_CR33","first-page":"141","volume":"14","author":"ET Collins","year":"1894","unstructured":"Collins ET (1894) Intra-ocular growths (two cases, brother and sister, with peculiar vascular new growth, probably retinal, affecting both eyes). Trans Ophthalmol Soc UK 14:141\u2013149","journal-title":"Trans Ophthalmol Soc UK"},{"issue":"5","key":"1091_CR34","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1101\/gad.1399906","volume":"20","author":"KL Covello","year":"2006","unstructured":"Covello KL, Kehler J, Yu H et al (2006) HIF-2alpha regulates Oct-4: effects of hypoxia on stem cell function, embryonic development, and tumor growth. Genes Dev 20(5):557\u2013570","journal-title":"Genes Dev"},{"issue":"2","key":"1091_CR35","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/j.ajhg.2007.10.006","volume":"82","author":"DW Craig","year":"2008","unstructured":"Craig DW, Itty A, Panganiban C et al (2008) Identification of somatic chromosomal abnormalities in hypothalamic hamartoma tissue at the GLI3 locus. Am J Hum Genet 82(2):366\u2013374","journal-title":"Am J Hum Genet"},{"issue":"13","key":"1091_CR36","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1056\/NEJMra055323","volume":"355","author":"PB Crino","year":"2006","unstructured":"Crino PB, Nathanson KL, Henske EP (2006) The tuberous sclerosis complex. N Engl J Med 355(13):1345\u20131356","journal-title":"N Engl J Med"},{"issue":"4","key":"1091_CR37","first-page":"407","volume":"5","author":"FF Cruz-Sanchez","year":"1990","unstructured":"Cruz-Sanchez FF, Rossi ML, Rodriguez-Prados S, Nakamura N, Hughes JT, Coakham HB (1990) Haemangioblastoma: histological and immunohistological study of an enigmatic cerebellar tumour. Histol Histopathol 5(4):407\u2013413","journal-title":"Histol Histopathol"},{"issue":"1","key":"1091_CR38","first-page":"1","volume":"101","author":"L Cuccurullo","year":"2009","unstructured":"Cuccurullo L, Prudente ME, Maffia S, Accardo M (2009) An ultrastructural study of the histogenesis of haemangioblastoma. Pathologica 101(1):1\u20135","journal-title":"Pathologica"},{"key":"1091_CR39","first-page":"447","volume":"57","author":"H Cushing","year":"1928","unstructured":"Cushing H, Bailey P (1928) Hemangiomas of cerebellum and retina (Lindau\u2019s disease). Arch Ophthalmol 57:447\u2013463","journal-title":"Arch Ophthalmol"},{"key":"1091_CR40","volume-title":"Tumors arising from the blood-vessels of the brain: angiomatous malformations and hemangioblastomas","author":"H Cushing","year":"1928","unstructured":"Cushing H, Bailey P (1928) Tumors arising from the blood-vessels of the brain: angiomatous malformations and hemangioblastomas. Springfield, Illinois"},{"issue":"3","key":"1091_CR41","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1128\/MCB.21.3.865-874.2001","volume":"21","author":"EJ Davidowitz","year":"2001","unstructured":"Davidowitz EJ, Schoenfeld AR, Burk RD (2001) VHL induces renal cell differentiation and growth arrest through integration of cell\u2013cell and cell-extracellular matrix signaling. Mol Cell Biol 21(3):865\u2013874","journal-title":"Mol Cell Biol"},{"issue":"5","key":"1091_CR42","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1227\/00006123-198911000-00002","volume":"25","author":"SM Monte de la","year":"1989","unstructured":"de la Monte SM, Horowitz SA (1989) Hemangioblastomas: clinical and histopathological factors correlated with recurrence. Neurosurgery 25(5):695\u2013698","journal-title":"Neurosurgery"},{"issue":"5","key":"1091_CR43","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1177\/1087057112436557","volume":"17","author":"Z Ding","year":"2012","unstructured":"Ding Z, German P, Bai S et al (2012) Agents that stabilize mutated von Hippel\u2013Lindau (VHL) protein: results of a high-throughput screen to identify compounds that modulate VHL proteostasis. J Biomol Screen 17(5):572\u2013580","journal-title":"J Biomol Screen"},{"issue":"4","key":"1091_CR44","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1677\/erc.1.00838","volume":"11","author":"G Eisenhofer","year":"2004","unstructured":"Eisenhofer G, Huynh TT, Pacak K et al (2004) Distinct gene expression profiles in norepinephrine- and epinephrine-producing hereditary and sporadic pheochromocytomas: activation of hypoxia-driven angiogenic pathways in von Hippel\u2013Lindau syndrome. Endocr Relat Cancer 11(4):897\u2013911","journal-title":"Endocr Relat Cancer"},{"issue":"3\u20134","key":"1091_CR45","doi-asserted-by":"crossref","first-page":"209","DOI":"10.3109\/00016488809097000","volume":"105","author":"C Erwall","year":"1988","unstructured":"Erwall C, Friberg U, Bagger-Sjoback D, Rask-Andersen H (1988) Degradation of the homogeneous substance in the endolymphatic sac. Acta Oto Laryngol 105(3\u20134):209\u2013217","journal-title":"Acta Oto Laryngol"},{"issue":"1","key":"1091_CR46","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1212\/WNL.41.1.41","volume":"41","author":"MR Filling-Katz","year":"1991","unstructured":"Filling-Katz MR, Choyke PL, Oldfield E et al (1991) Central nervous system involvement in Von Hippel\u2013Lindau disease. Neurology 41(1):41\u201346","journal-title":"Neurology"},{"issue":"6","key":"1091_CR47","first-page":"638","volume":"2","author":"TS Frank","year":"1989","unstructured":"Frank TS, Trojanowski JQ, Roberts SA, Brooks JJ (1989) A detailed immunohistochemical analysis of cerebellar hemangioblastoma: an undifferentiated mesenchymal tumor. Mod Pathol 2(6):638\u2013651","journal-title":"Mod Pathol"},{"issue":"6","key":"1091_CR48","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1016\/j.ceb.2007.10.001","volume":"19","author":"IJ Frew","year":"2007","unstructured":"Frew IJ, Krek W (2007) Multitasking by pVHL in tumour suppression. Curr Opin Cell Biol 19(6):685\u2013690","journal-title":"Curr Opin Cell Biol"},{"issue":"12","key":"1091_CR49","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1038\/emboj.2008.96","volume":"27","author":"IJ Frew","year":"2008","unstructured":"Frew IJ, Thoma CR, Georgiev S et al (2008) pVHL and PTEN tumour suppressor proteins cooperatively suppress kidney cyst formation. EMBO J 27(12):1747\u20131757","journal-title":"EMBO J"},{"issue":"7","key":"1091_CR50","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1001\/archotol.1984.00800330003001","volume":"110","author":"U Friberg","year":"1984","unstructured":"Friberg U, Rask-Andersen H, Bagger-Sjoback D (1984) Human endolymphatic duct. An ultrastructural study. Arch Otolaryngol 110(7):421\u2013428","journal-title":"Arch Otolaryngol"},{"issue":"14","key":"1091_CR51","doi-asserted-by":"crossref","first-page":"4029","DOI":"10.1093\/emboj\/17.14.4029","volume":"17","author":"M Gering","year":"1998","unstructured":"Gering M, Rodaway AR, Gottgens B, Patient RK, Green AR (1998) The SCL gene specifies haemangioblast development from early mesoderm. EMBO J 17(14):4029\u20134045","journal-title":"EMBO J"},{"issue":"5","key":"1091_CR52","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1136\/jnnp.70.5.644","volume":"70","author":"S Glasker","year":"2001","unstructured":"Glasker S, Bender BU, Apel TW et al (2001) Reconsideration of biallelic inactivation of the VHL tumour suppressor gene in hemangioblastomas of the central nervous system. J Neurol Neurosurg Psychiatry 70(5):644\u2013648","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"8","key":"1091_CR53","doi-asserted-by":"crossref","first-page":"4167","DOI":"10.1158\/0008-5472.CAN-05-3505","volume":"66","author":"S Glasker","year":"2006","unstructured":"Glasker S, Li J, Xia JB et al (2006) Hemangioblastomas share protein expression with embryonal hemangioblast progenitor cell. Cancer Res 66(8):4167\u20134172","journal-title":"Cancer Res"},{"issue":"23","key":"1091_CR54","doi-asserted-by":"crossref","first-page":"10847","DOI":"10.1158\/0008-5472.CAN-05-1104","volume":"65","author":"S Glasker","year":"2005","unstructured":"Glasker S, Lonser RR, Tran MG et al (2005) Effects of VHL deficiency on endolymphatic duct and sac. Cancer Res 65(23):10847\u201310853","journal-title":"Cancer Res"},{"issue":"1","key":"1091_CR55","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1002\/path.2029","volume":"210","author":"S Glasker","year":"2006","unstructured":"Glasker S, Tran MG, Shively SB et al (2006) Epididymal cystadenomas and epithelial tumorlets: effects of VHL deficiency on human epididymis. J Pathol 210(1):32\u201341","journal-title":"J Pathol"},{"issue":"1","key":"1091_CR56","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1227\/01.NEU.0000163250.71951.18","volume":"57","author":"S Glasker","year":"2005","unstructured":"Glasker S, van Velthoven V (2005) Risk of hemorrhage in hemangioblastomas of the central nervous system. Neurosurgery 57(1):71\u201376","journal-title":"Neurosurgery"},{"issue":"17","key":"1091_CR57","doi-asserted-by":"crossref","first-page":"9102","DOI":"10.1073\/pnas.94.17.9102","volume":"94","author":"JR Gnarra","year":"1997","unstructured":"Gnarra JR, Ward JM, Porter FD et al (1997) Defective placental vasculogenesis causes embryonic lethality in VHL-deficient mice. Proc Natl Acad Sci USA 94(17):9102\u20139107","journal-title":"Proc Natl Acad Sci USA"},{"issue":"8","key":"1091_CR58","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1136\/jcp.41.8.861","volume":"41","author":"DR Gouldesbrough","year":"1988","unstructured":"Gouldesbrough DR, Bell JE, Gordon A (1988) Use of immunohistochemical methods in the differential diagnosis between primary cerebellar haemangioblastoma and metastatic renal carcinoma. J Clin Pathol 41(8):861\u2013865","journal-title":"J Clin Pathol"},{"issue":"5","key":"1091_CR59","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1002\/stem.5530100504","volume":"10","author":"AR Green","year":"1992","unstructured":"Green AR, Begley CG (1992) SCL and related hemopoietic helix-loop-helix transcription factors. Int J Cell Cloning 10(5):269\u2013276","journal-title":"Int J Cell Cloning"},{"issue":"3","key":"1091_CR60","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/0090-3019(84)90010-7","volume":"22","author":"F Grisoli","year":"1984","unstructured":"Grisoli F, Gambarelli D, Raybaud C, Guibout M, Leclercq T (1984) Suprasellar hemangioblastoma. Surg Neurol 22(3):257\u2013262","journal-title":"Surg Neurol"},{"issue":"5","key":"1091_CR61","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/j.devcel.2005.09.010","volume":"9","author":"MV Gustafsson","year":"2005","unstructured":"Gustafsson MV, Zheng X, Pereira T et al (2005) Hypoxia requires notch signaling to maintain the undifferentiated cell state. Dev Cell 9(5):617\u2013628","journal-title":"Dev Cell"},{"issue":"4\u20135","key":"1091_CR62","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1016\/j.semcdb.2005.03.006","volume":"16","author":"VH Haase","year":"2005","unstructured":"Haase VH (2005) The VHL tumor suppressor in development and disease: functional studies in mice by conditional gene targeting. Semin Cell Dev Biol 16(4\u20135):564\u2013574","journal-title":"Semin Cell Dev Biol"},{"issue":"12","key":"1091_CR63","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1046\/j.1440-1827.2001.01298.x","volume":"51","author":"S Hamazaki","year":"2001","unstructured":"Hamazaki S, Nakashima H, Matsumoto K, Taguchi K, Okada S (2001) Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von Hippel\u2013Lindau disease. Pathol Int 51(12):948\u2013953","journal-title":"Pathol Int"},{"issue":"4","key":"1091_CR64","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.1053\/gast.2000.18143","volume":"119","author":"PR Hammel","year":"2000","unstructured":"Hammel PR, Vilgrain V, Terris B et al (2000) Pancreatic involvement in von Hippel\u2013Lindau disease. The Groupe Francophone d\u2019Etude de la Maladie de von Hippel\u2013Lindau. Gastroenterology 119(4):1087\u20131095","journal-title":"Gastroenterology"},{"issue":"3","key":"1091_CR65","first-page":"193","volume":"10","author":"JP Harris","year":"1989","unstructured":"Harris JP (1989) Autoimmunity of the inner ear. Am J Otol 10(3):193\u2013195","journal-title":"Am J Otol"},{"key":"1091_CR66","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1111\/j.1365-2990.2005.00669.x","volume":"31","author":"M Hasselblatt","year":"2005","unstructured":"Hasselblatt M, Jeibmann A, Ger\u00df J et al (2005) Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. Neuropathol Appl Neurobiol 31:618\u2013622","journal-title":"Neuropathol Appl Neurobiol"},{"issue":"11","key":"1091_CR67","doi-asserted-by":"crossref","first-page":"2292","DOI":"10.1002\/1097-0142(19891201)64:11<2292::AID-CNCR2820641119>3.0.CO;2-#","volume":"64","author":"DK Heffner","year":"1989","unstructured":"Heffner DK (1989) Low-grade adenocarcinoma of probable endolymphatic sac origin: a clinicopathologic study of 20 cases. Cancer 64(11):2292\u20132302","journal-title":"Cancer"},{"issue":"3","key":"1091_CR68","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1210\/jc.2002-021466","volume":"88","author":"FJ Hes","year":"2003","unstructured":"Hes FJ, Hoppener JW, Lips CJ (2003) Clinical review 155: pheochromocytoma in Von Hippel\u2013Lindau disease. J Clin Endocrinol Metab 88(3):969\u2013974","journal-title":"J Clin Endocrinol Metab"},{"key":"1091_CR69","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1097\/00005072-198411000-00004","volume":"43","author":"K Ho","year":"1984","unstructured":"Ho K (1984) Ultrastructure of cerebellar capillary hemangioblastoma. I. Weibel\u2013Palade bodies and stroma cell histogenesis. J Neuropathol Exp Neurol 43:592\u2013608","journal-title":"J Neuropathol Exp Neurol"},{"issue":"8","key":"1091_CR70","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1097\/00000478-200308000-00014","volume":"27","author":"MP Hoang","year":"2003","unstructured":"Hoang MP, Amirkhan RH (2003) Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol 27(8):1152\u20131156","journal-title":"Am J Surg Pathol"},{"issue":"4","key":"1091_CR71","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1093\/ajcp\/86.4.423","volume":"86","author":"SC Holt","year":"1986","unstructured":"Holt SC, Bruner JM, Ordonez NG (1986) Capillary hemangioblastoma. An immunohistochemical study. Am J Clin Pathol 86(4):423\u2013429","journal-title":"Am J Clin Pathol"},{"issue":"3","key":"1091_CR72","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1097\/00000478-198903000-00005","volume":"13","author":"TJ Hufnagel","year":"1989","unstructured":"Hufnagel TJ, Kim JH, True LD, Manuelidis EE (1989) Immunohistochemistry of capillary hemangioblastoma. Immunoperoxidase-labeled antibody staining resolves the differential diagnosis with metastatic renal cell carcinoma, but does not explain the histogenesis of the capillary hemangioblastoma. Am J Surg Pathol 13(3):207\u2013216","journal-title":"Am J Surg Pathol"},{"issue":"20","key":"1091_CR73","doi-asserted-by":"crossref","first-page":"10595","DOI":"10.1073\/pnas.93.20.10595","volume":"93","author":"O Iliopoulos","year":"1996","unstructured":"Iliopoulos O, Levy AP, Jiang C, Kaelin WG Jr, Goldberg MA (1996) Negative regulation of hypoxia-inducible genes by the von Hippel\u2013Lindau protein. Proc Natl Acad Sci USA 93(20):10595\u201310599","journal-title":"Proc Natl Acad Sci USA"},{"issue":"6","key":"1091_CR74","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1111\/j.1365-2559.2008.03003.x","volume":"52","author":"B Ingold","year":"2008","unstructured":"Ingold B, Wild PJ, Nocito A et al (2008) Renal cell carcinoma marker reliably discriminates central nervous system haemangioblastoma from brain metastases of renal cell carcinoma. Histopathology 52(6):674\u2013681","journal-title":"Histopathology"},{"key":"1091_CR75","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1111\/j.1365-2559.1988.tb01914.x","volume":"12","author":"JW Ironside","year":"1988","unstructured":"Ironside JW, Stephenson TJ, Royds JA et al (1988) Stromal cells in cerebellar hemangioblastomas: an immunocytochemical study. Histopathology 12:29\u201340","journal-title":"Histopathology"},{"issue":"7","key":"1091_CR76","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1111\/j.1440-1827.2005.01841.x","volume":"55","author":"K Ishizawa","year":"2005","unstructured":"Ishizawa K, Komori T, Hirose T (2005) Stromal cells in hemangioblastoma: neuroectodermal differentiation and morphological similarities to ependymoma. Pathol Int 55(7):377\u2013385","journal-title":"Pathol Int"},{"key":"1091_CR77","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1136\/jcp.38.4.417","volume":"38","author":"SM Ismail","year":"1985","unstructured":"Ismail SM, Jasani B, Cole G (1985) Histogenesis of hemangioblastomas: an immunocytochemical and ultrastructural study in a case of von Hippel\u2013Lindau syndrome. J Clin Pathol 38:417\u2013421","journal-title":"J Clin Pathol"},{"issue":"3","key":"1091_CR78","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1007\/BF00345942","volume":"15","author":"A Jamjoom","year":"1992","unstructured":"Jamjoom A, Kane N, Nicoll J (1992) Metastasis of a renal carcinoma to a cerebellar haemangioblastoma in a case of von Hippel\u2013Lindau disease. Neurosurg Rev 15(3):231\u2013234","journal-title":"Neurosurg Rev"},{"issue":"2","key":"1091_CR79","doi-asserted-by":"crossref","first-page":"256","DOI":"10.3171\/jns.2006.105.2.256","volume":"105","author":"ST Jarrell","year":"2006","unstructured":"Jarrell ST, Vortmeyer AO, Linehan WM, Oldfield EH, Lonser RR (2006) Metastases to hemangioblastomas in von Hippel\u2013Lindau disease. J Neurosurg 105(2):256\u2013263","journal-title":"J Neurosurg"},{"issue":"31","key":"1091_CR80","doi-asserted-by":"crossref","first-page":"19253","DOI":"10.1074\/jbc.272.31.19253","volume":"272","author":"BH Jiang","year":"1997","unstructured":"Jiang BH, Zheng JZ, Leung SW, Roe R, Semenza GL (1997) Transactivation and inhibitory domains of hypoxia-inducible factor 1alpha. Modulation of transcriptional activity by oxygen tension. J Biol Chem 272(31):19253\u201319260","journal-title":"J Biol Chem"},{"issue":"1","key":"1091_CR81","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/S1470-2045(07)70411-4","volume":"9","author":"J Jozwiak","year":"2008","unstructured":"Jozwiak J, Jozwiak S, Wlodarski P (2008) Possible mechanisms of disease development in tuberous sclerosis. Lancet Oncol 9(1):73\u201379","journal-title":"Lancet Oncol"},{"issue":"6","key":"1091_CR82","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1038\/modpathol.3800351","volume":"18","author":"SM Jung","year":"2005","unstructured":"Jung SM, Kuo TT (2005) Immunoreactivity of CD10 and inhibin alpha in differentiating hemangioblastoma of central nervous system from metastatic clear cell renal cell carcinoma. Mod Pathol 18(6):788\u2013794","journal-title":"Mod Pathol"},{"issue":"1","key":"1091_CR83","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0046-8177(82)80133-0","volume":"13","author":"S Jurco 3rd","year":"1982","unstructured":"Jurco S 3rd, Nadji M, Harvey DG, Parker JC Jr, Font RL, Morales AR (1982) Hemangioblastomas: histogenesis of the stromal cell studied by immunocytochemistry. Hum Pathol 13(1):13\u201318","journal-title":"Hum Pathol"},{"issue":"9","key":"1091_CR84","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1038\/nrc885","volume":"2","author":"WG Kaelin Jr","year":"2002","unstructured":"Kaelin WG Jr (2002) Molecular basis of the VHL hereditary cancer syndrome. Nat Rev Cancer 2(9):673\u2013682","journal-title":"Nat Rev Cancer"},{"issue":"Suppl 10","key":"1091_CR85","doi-asserted-by":"crossref","first-page":"2262","DOI":"10.1002\/cncr.24232","volume":"115","author":"WG Kaelin Jr","year":"2009","unstructured":"Kaelin WG Jr (2009) Treatment of kidney cancer: insights provided by the VHL tumor-suppressor protein. Cancer 115(Suppl 10):2262\u20132272","journal-title":"Cancer"},{"issue":"4","key":"1091_CR86","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1007\/BF00688262","volume":"73","author":"H Kamitani","year":"1987","unstructured":"Kamitani H, Masuzawa H, Sato J, Kanazawa I (1987) Capillary hemangioblastoma: histogenesis of stromal cells. Acta Neuropathol (Berl) 73(4):370\u2013378","journal-title":"Acta Neuropathol (Berl)"},{"issue":"6","key":"1091_CR87","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1016\/S1097-2765(01)00258-1","volume":"7","author":"P Karuman","year":"2001","unstructured":"Karuman P, Gozani O, Odze RD et al (2001) The Peutz\u2013Jegher gene product LKB1 is a mediator of p53-dependent cell death. Mol Cell 7(6):1307\u20131319","journal-title":"Mol Cell"},{"issue":"1","key":"1091_CR88","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1212\/WNL.53.1.208","volume":"53","author":"N Kawahara","year":"1999","unstructured":"Kawahara N, Kume H, Ueki K, Mishima K, Sasaki T, Kirino T (1999) VHL gene inactivation in an endolymphatic sac tumor associated with von Hippel\u2013Lindau disease. Neurology 53(1):208\u2013210","journal-title":"Neurology"},{"issue":"6","key":"1091_CR89","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.1002\/1097-0142(197306)31:6<1528::AID-CNCR2820310634>3.0.CO;2-R","volume":"31","author":"J Kawamura","year":"1973","unstructured":"Kawamura J, Garcia JH, Kamijyo Y (1973) Cerebellar hemangioblastoma: histogenesis of stroma cells. Cancer 31(6):1528\u20131540","journal-title":"Cancer"},{"issue":"1","key":"1091_CR90","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1046\/j.1365-2559.1998.00460.x","volume":"33","author":"G Kempermann","year":"1998","unstructured":"Kempermann G, Neumann HP, Volk B (1998) Endolymphatic sac tumours. Histopathology 33(1):2\u201310","journal-title":"Histopathology"},{"issue":"3","key":"1091_CR91","doi-asserted-by":"crossref","first-page":"503","DOI":"10.3171\/jns.2005.102.3.0503","volume":"102","author":"HJ Kim","year":"2005","unstructured":"Kim HJ, Butman JA, Brewer C et al (2005) Tumors of the endolymphatic sac in patients with von Hippel\u2013Lindau disease: implications for their natural history, diagnosis, and treatment. J Neurosurg 102(3):503\u2013512","journal-title":"J Neurosurg"},{"issue":"3","key":"1091_CR92","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1007\/BF01366952","volume":"122","author":"AG Knudson","year":"1996","unstructured":"Knudson AG (1996) Hereditary cancer: two hits revisited. J Cancer Res Clin Oncol 122(3):135\u2013140","journal-title":"J Cancer Res Clin Oncol"},{"issue":"1","key":"1091_CR93","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1385\/EP:13:1:17","volume":"13","author":"CA Koch","year":"2002","unstructured":"Koch CA, Mauro D, Walther MM et al (2002) Pheochromocytoma in von Hippel\u2013Lindau disease: distinct histopathologic phenotype compared to pheochromocytoma in multiple endocrine neoplasia type 2. Endocr Pathol 13(1):17\u201327","journal-title":"Endocr Pathol"},{"issue":"1","key":"1091_CR94","first-page":"43","volume":"35","author":"CA Koch","year":"2001","unstructured":"Koch CA, Vortmeyer AO, Huang SC, Alesci S, Zhuang Z, Pacak K (2001) Genetic aspects of pheochromocytoma. Endocr Regul 35(1):43\u201352","journal-title":"Endocr Regul"},{"issue":"1","key":"1091_CR95","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/j.clindermatol.2004.09.007","volume":"23","author":"BR Korf","year":"2005","unstructured":"Korf BR (2005) The phakomatoses. Clin Dermatol 23(1):78\u201384","journal-title":"Clin Dermatol"},{"issue":"48","key":"1091_CR96","doi-asserted-by":"crossref","first-page":"5435","DOI":"10.1038\/sj.onc.1203938","volume":"19","author":"M Krieg","year":"2000","unstructured":"Krieg M, Haas R, Brauch H, Acker T, Flamme I, Plate KH (2000) Up-regulation of hypoxia-inducible factors HIF-1alpha and HIF-2alpha under normoxic conditions in renal carcinoma cells by von Hippel\u2013Lindau tumor suppressor gene loss of function. Oncogene 19(48):5435\u20135443","journal-title":"Oncogene"},{"issue":"5","key":"1091_CR97","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1080\/019131299281446","volume":"23","author":"B Lach","year":"1999","unstructured":"Lach B, Gregor A, Rippstein P, Omulecka A (1999) Angiogenic histogenesis of stromal cells in hemangioblastoma: ultrastructural and immunohistochemical study. Ultrastruct Pathol 23(5):299\u2013310","journal-title":"Ultrastruct Pathol"},{"issue":"1","key":"1091_CR98","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/00005792-198901000-00001","volume":"68","author":"JM Lamiell","year":"1989","unstructured":"Lamiell JM, Salazar FG, Hsia YE (1989) von Hippel\u2013Lindau disease affecting 43 members of a single kindred. Medicine (Baltimore) 68(1):1\u201329","journal-title":"Medicine (Baltimore)"},{"issue":"5112","key":"1091_CR99","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1126\/science.8493574","volume":"260","author":"F Latif","year":"1993","unstructured":"Latif F, Tory K, Gnarra J et al (1993) Identification of the von Hippel\u2013Lindau disease tumor suppressor gene. Science 260(5112):1317\u20131320","journal-title":"Science"},{"issue":"3","key":"1091_CR100","first-page":"504","volume":"58","author":"JY Lee","year":"1998","unstructured":"Lee JY, Dong SM, Park WS et al (1998) Loss of heterozygosity and somatic mutations of the VHL tumor suppressor gene in sporadic cerebellar hemangioblastomas. Cancer Res 58(3):504\u2013508","journal-title":"Cancer Res"},{"issue":"6","key":"1091_CR101","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1067\/msy.1998.91823","volume":"124","author":"SK Libutti","year":"1998","unstructured":"Libutti SK, Choyke PL, Bartlett DL et al (1998) Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease: diagnostic and management recommendations. Surgery 124(6):1153\u20131159","journal-title":"Surgery"},{"key":"1091_CR102","first-page":"1","volume":"1","author":"A Lindau","year":"1926","unstructured":"Lindau A (1926) Studien uber Kleinhirnzysten. Bau, Pathogenese und Beziehung zur Angiomatosis retinae. Acta Path et Microbiol Scand Suppl 1:1\u2013126","journal-title":"Acta Path et Microbiol Scand Suppl"},{"issue":"14","key":"1091_CR103","doi-asserted-by":"crossref","first-page":"1822","DOI":"10.1101\/gad.13.14.1822","volume":"13","author":"J Lisztwan","year":"1999","unstructured":"Lisztwan J, Imbert G, Wirbelauer C, Gstaiger M, Krek W (1999) The von Hippel\u2013Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity. Genes Dev 13(14):1822\u20131833","journal-title":"Genes Dev"},{"key":"1091_CR104","unstructured":"Lloyd RV, Tischer AS, Kimura N, McNicol AM, Young WF (2004) Von Hippel\u2013Lindau syndrome (VHL). In: DeLellis RA, Lloyd RV, Heitz PU, Eng C (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of endocrine organs. IARC Press, Lyon, pp 137\u2013146"},{"issue":"5","key":"1091_CR105","first-page":"881","volume":"18","author":"WW Lo","year":"1997","unstructured":"Lo WW, Daniels DL, Chakeres DW et al (1997) The endolymphatic duct and sac. Am J Neuroradiol 18(5):881\u2013887","journal-title":"Am J Neuroradiol"},{"issue":"2","key":"1091_CR106","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1128\/MCB.18.2.732","volume":"18","author":"KM Lonergan","year":"1998","unstructured":"Lonergan KM, Iliopoulos O, Ohh M et al (1998) Regulation of hypoxia-inducible mRNAs by the von Hippel\u2013Lindau tumor suppressor protein requires binding to complexes containing elongins B\/C and Cul2. Mol Cell Biol 18(2):732\u2013741","journal-title":"Mol Cell Biol"},{"issue":"9374","key":"1091_CR107","doi-asserted-by":"crossref","first-page":"2059","DOI":"10.1016\/S0140-6736(03)13643-4","volume":"361","author":"RR Lonser","year":"2003","unstructured":"Lonser RR, Glenn GM, Walther M et al (2003) von Hippel\u2013Lindau disease. Lancet 361(9374):2059\u20132067","journal-title":"Lancet"},{"issue":"24","key":"1091_CR108","doi-asserted-by":"crossref","first-page":"2481","DOI":"10.1056\/NEJMoa040666","volume":"350","author":"RR Lonser","year":"2004","unstructured":"Lonser RR, Kim HJ, Butman JA, Vortmeyer AO, Choo DI, Oldfield EH (2004) Tumors of the endolymphatic sac in von Hippel\u2013Lindau disease. N Engl J Med 350(24):2481\u20132486","journal-title":"N Engl J Med"},{"key":"1091_CR109","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1002\/ana.20584","volume":"58","author":"RR Lonser","year":"2005","unstructured":"Lonser RR, Vortmeyer AO, Butman JA et al (2005) Edema is a precursor to central nervous system peritumoral cyst formation. Ann Neurol 58:392\u2013399","journal-title":"Ann Neurol"},{"issue":"3","key":"1091_CR110","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1002\/ana.20584","volume":"58","author":"RR Lonser","year":"2005","unstructured":"Lonser RR, Vortmeyer AO, Butman JA et al (2005) Edema is a precursor to central nervous system peritumoral cyst formation. Ann Neurol 58(3):392\u2013399","journal-title":"Ann Neurol"},{"issue":"1","key":"1091_CR111","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/S0002-9440(10)65563-0","volume":"153","author":"IA Lubensky","year":"1998","unstructured":"Lubensky IA, Pack S, Ault D et al (1998) Multiple neuroendocrine tumors of the pancreas in von Hippel\u2013Lindau disease patients: histopathological and molecular genetic analysis. Am J Pathol 153(1):223\u2013231","journal-title":"Am J Pathol"},{"issue":"1","key":"1091_CR112","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1093\/carcin\/bgq214","volume":"32","author":"D Ma","year":"2011","unstructured":"Ma D, Zhang M, Chen L et al (2011) Hemangioblastomas might derive from neoplastic transformation of neural stem cells\/progenitors in the specific niche. Carcinogenesis 32(1):102\u2013109","journal-title":"Carcinogenesis"},{"issue":"1","key":"1091_CR113","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/S1535-6108(02)00240-4","volume":"3","author":"FA Mack","year":"2003","unstructured":"Mack FA, Rathmell WK, Arsham AM, Gnarra J, Keith B, Simon MC (2003) Loss of pVHL is sufficient to cause HIF dysregulation in primary cells but does not promote tumor growth. Cancer Cell 3(1):75\u201388","journal-title":"Cancer Cell"},{"issue":"7","key":"1091_CR114","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1136\/jmg.28.7.443","volume":"28","author":"ER Maher","year":"1991","unstructured":"Maher ER, Iselius L, Yates JR et al (1991) Von Hippel\u2013Lindau disease: a genetic study. J Med Genet 28(7):443\u2013447","journal-title":"J Med Genet"},{"issue":"283","key":"1091_CR115","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1093\/qjmed\/77.2.1151","volume":"77","author":"ER Maher","year":"1990","unstructured":"Maher ER, Yates JR, Harries R et al (1990) Clinical features and natural history of von Hippel\u2013Lindau disease. Q J Med 77(283):1151\u20131163","journal-title":"Q J Med"},{"key":"1091_CR116","unstructured":"Majno G, Joris I (2004) Cells, tissue, and disease: principles of general pathology. Oxford University Press, NY"},{"key":"1091_CR117","first-page":"2225","volume-title":"Hypertension: pathophysiology, diagnosis and management","author":"WM Manger","year":"1995","unstructured":"Manger WM, Gifford RW Jr (1995) Pheochromocytoma: a clinical overview. In: Laragh JH, Brenner BM (eds) Hypertension: pathophysiology, diagnosis and management. Raven Press, New York, pp 2225\u20132244"},{"issue":"18","key":"1091_CR118","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1001\/jama.1997.03540420057030","volume":"277","author":"TJ Manski","year":"1997","unstructured":"Manski TJ, Heffner DK, Glenn GM et al (1997) Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel\u2013Lindau disease. JAMA 277(18):1461\u20131466","journal-title":"JAMA"},{"issue":"18","key":"1091_CR119","doi-asserted-by":"crossref","first-page":"5197","DOI":"10.1093\/emboj\/20.18.5197","volume":"20","author":"N Masson","year":"2001","unstructured":"Masson N, Willam C, Maxwell PH, Pugh CW, Ratcliffe PJ (2001) Independent function of two destruction domains in hypoxia-inducible factor-alpha chains activated by prolyl hydroxylation. EMBO J 20(18):5197\u20135206","journal-title":"EMBO J"},{"issue":"6733","key":"1091_CR120","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1038\/20459","volume":"399","author":"PH Maxwell","year":"1999","unstructured":"Maxwell PH, Wiesener MS, Chang GW et al (1999) The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis. Nature 399(6733):271\u2013275","journal-title":"Nature"},{"issue":"5","key":"1091_CR121","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.cell.2005.03.024","volume":"121","author":"AJ McClellan","year":"2005","unstructured":"McClellan AJ, Scott MD, Frydman J (2005) Folding and quality control of the VHL tumor suppressor proceed through distinct chaperone pathways. Cell 121(5):739\u2013748","journal-title":"Cell"},{"key":"1091_CR122","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/0002-9343(64)90107-X","volume":"36","author":"KL Melmon","year":"1964","unstructured":"Melmon KL, Rosen SW (1964) Lindau\u2019s disease. Review of the literature and study of a large kindred. Am J Med 36:595\u2013617","journal-title":"Am J Med"},{"issue":"2","key":"1091_CR123","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1007\/s12105-007-0016-3","volume":"1","author":"L Michaels","year":"2007","unstructured":"Michaels L (2007) Origin of endolymphatic sac tumor. Head Neck Pathol 1(2):104\u2013111","journal-title":"Head Neck Pathol"},{"issue":"5","key":"1091_CR124","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/S0196-0709(82)80004-5","volume":"3","author":"C Morgenstern","year":"1982","unstructured":"Morgenstern C, Amano H, Orsulakova A (1982) Ion transport in the endolymphatic space. Am J Otolaryngol 3(5):323\u2013327","journal-title":"Am J Otolaryngol"},{"issue":"10","key":"1091_CR125","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1007\/s007010170012","volume":"143","author":"C Mottolese","year":"2001","unstructured":"Mottolese C, Stan H, Giordano F, Frappaz D, Alexei D, Streichenberger N (2001) Metastasis of clear-cell renal carcinoma to cerebellar hemangioblastoma in von Hippel Lindau disease: rare or not investigated? Acta Neurochir (Wien) 143(10):1059\u20131063","journal-title":"Acta Neurochir (Wien)"},{"issue":"7","key":"1091_CR126","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1016\/0046-8177(92)90351-3","volume":"23","author":"Z Nemes","year":"1992","unstructured":"Nemes Z (1992) Fibrohistiocytic differentiation in capillary hemangioblastoma. Hum Pathol 23(7):805\u2013810","journal-title":"Hum Pathol"},{"key":"1091_CR127","volume-title":"Atlas of human anatomy","author":"FH Netter","year":"1989","unstructured":"Netter FH, Colacino S (1989) Atlas of human anatomy. Ciba Geigy Corporation, Summit"},{"issue":"4","key":"1091_CR128","first-page":"309","volume":"16","author":"HP Neumann","year":"1987","unstructured":"Neumann HP (1987) Prognosis of von Hippel\u2013Lindau syndrome. Vasa 16(4):309\u2013311","journal-title":"Vasa"},{"issue":"19","key":"1091_CR129","doi-asserted-by":"crossref","first-page":"1459","DOI":"10.1056\/NEJMoa020152","volume":"346","author":"HP Neumann","year":"2002","unstructured":"Neumann HP, Bausch B, McWhinney SR et al (2002) Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 346(19):1459\u20131466","journal-title":"N Engl J Med"},{"issue":"21","key":"1091_CR130","doi-asserted-by":"crossref","first-page":"1531","DOI":"10.1056\/NEJM199311183292103","volume":"329","author":"HP Neumann","year":"1993","unstructured":"Neumann HP, Berger DP, Sigmund G et al (1993) Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel\u2013Lindau disease. N Engl J Med 329(21):1531\u20131538","journal-title":"N Engl J Med"},{"issue":"1","key":"1091_CR131","doi-asserted-by":"crossref","first-page":"24","DOI":"10.3171\/jns.1989.70.1.0024","volume":"70","author":"HP Neumann","year":"1989","unstructured":"Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P (1989) Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel\u2013Lindau syndrome. J Neurosurg 70(1):24\u201330","journal-title":"J Neurosurg"},{"issue":"8761","key":"1091_CR132","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/0140-6736(91)90401-A","volume":"338","author":"HP Neumann","year":"1991","unstructured":"Neumann HP, Wiestler OD (1991) Clustering of features and genetics of von Hippel\u2013Lindau syndrome. Lancet 338(8761):258","journal-title":"Lancet"},{"issue":"2","key":"1091_CR133","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1007\/BF00453767","volume":"243","author":"O Ninoyu","year":"1986","unstructured":"Ninoyu O, Meyer zum Gottesberge AM (1986) Ca++ activity in the endolymphatic space. Arch Otorhinolaryngol 243(2):141\u2013142","journal-title":"Arch Otorhinolaryngol"},{"issue":"4","key":"1091_CR134","doi-asserted-by":"crossref","first-page":"2060","DOI":"10.1074\/jbc.274.4.2060","volume":"274","author":"JF O\u2019Rourke","year":"1999","unstructured":"O\u2019Rourke JF, Tian YM, Ratcliffe PJ, Pugh CW (1999) Oxygen-regulated and transactivating domains in endothelial PAS protein 1: comparison with hypoxia-inducible factor-1alpha. J Biol Chem 274(4):2060\u20132071","journal-title":"J Biol Chem"},{"issue":"7","key":"1091_CR135","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1038\/35017054","volume":"2","author":"M Ohh","year":"2000","unstructured":"Ohh M, Park CW, Ivan M et al (2000) Ubiquitination of hypoxia-inducible factor requires direct binding to the beta-domain of the von Hippel\u2013Lindau protein. Nat Cell Biol 2(7):423\u2013427","journal-title":"Nat Cell Biol"},{"issue":"1","key":"1091_CR136","first-page":"41","volume":"33","author":"A Omulecka","year":"1995","unstructured":"Omulecka A, Lach B, Alwasiak J, Gregor A (1995) Immunohistochemical and ultrastructural studies of stromal cells in hemangioblastoma. Folia Neuropathol 33(1):41\u201350","journal-title":"Folia Neuropathol"},{"issue":"6","key":"1091_CR137","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1016\/j.beem.2010.05.001","volume":"24","author":"G Opocher","year":"2010","unstructured":"Opocher G, Schiavi F (2010) Genetics of pheochromocytomas and paragangliomas. Best Pract Res Clin Endocrinol Metab 24(6):943\u2013956","journal-title":"Best Pract Res Clin Endocrinol Metab"},{"issue":"3","key":"1091_CR138","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1002\/jcp.21812","volume":"220","author":"DM Panchision","year":"2009","unstructured":"Panchision DM (2009) The role of oxygen in regulating neural stem cells in development and disease. J Cell Physiol 220(3):562\u2013568","journal-title":"J Cell Physiol"},{"issue":"2","key":"1091_CR139","doi-asserted-by":"crossref","first-page":"e60","DOI":"10.1371\/journal.pmed.0040060","volume":"4","author":"DM Park","year":"2007","unstructured":"Park DM, Zhuang Z, Chen L et al (2007) von Hippel\u2013Lindau disease-associated hemangioblastomas are derived from embryologic multipotent cells. PLoS Med 4(2):e60","journal-title":"PLoS Med"},{"issue":"4","key":"1091_CR140","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1038\/cdd.2008.17","volume":"15","author":"SA Patel","year":"2008","unstructured":"Patel SA, Simon MC (2008) Biology of hypoxia-inducible factor-2alpha in development and disease. Cell Death Differ 15(4):628\u2013634","journal-title":"Cell Death Differ"},{"issue":"6","key":"1091_CR141","doi-asserted-by":"crossref","first-page":"2156","DOI":"10.1073\/pnas.94.6.2156","volume":"94","author":"A Pause","year":"1997","unstructured":"Pause A, Lee S, Worrell RA et al (1997) The von Hippel\u2013Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins. Proc Natl Acad Sci USA 94(6):2156\u20132161","journal-title":"Proc Natl Acad Sci USA"},{"issue":"8","key":"1091_CR142","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1177\/000348949510400805","volume":"104","author":"A Pollak","year":"1995","unstructured":"Pollak A, Bohmer A, Spycher M, Fisch U (1995) Are papillary adenomas endolymphatic sac tumors? Ann Otol Rhinol Laryngol 104(8):613\u2013619","journal-title":"Ann Otol Rhinol Laryngol"},{"issue":"4","key":"1091_CR143","doi-asserted-by":"crossref","first-page":"641","DOI":"10.5858\/2007-131-641-MRCCTH","volume":"131","author":"AD Polydorides","year":"2007","unstructured":"Polydorides AD, Rosenblum MK, Edgar MA (2007) Metastatic renal cell carcinoma to hemangioblastoma in von Hippel\u2013Lindau disease. Arch Pathol Lab Med 131(4):641\u2013645","journal-title":"Arch Pathol Lab Med"},{"issue":"4","key":"1091_CR144","first-page":"765","volume":"60","author":"AH Prowse","year":"1997","unstructured":"Prowse AH, Webster AR, Richards FM et al (1997) Somatic inactivation of the VHL gene in Von Hippel\u2013Lindau disease tumors. Am J Hum Genet 60(4):765\u2013771","journal-title":"Am J Hum Genet"},{"issue":"5","key":"1091_CR145","doi-asserted-by":"crossref","first-page":"2576","DOI":"10.1158\/0008-5472.CAN-05-3241","volume":"66","author":"EB Rankin","year":"2006","unstructured":"Rankin EB, Tomaszewski JE, Haase VH (2006) Renal cyst development in mice with conditional inactivation of the von Hippel\u2013Lindau tumor suppressor. Cancer Res 66(5):2576\u20132583","journal-title":"Cancer Res"},{"issue":"3\u20134","key":"1091_CR146","doi-asserted-by":"crossref","first-page":"283","DOI":"10.3109\/00016488009127140","volume":"89","author":"H Rask-Andersen","year":"1980","unstructured":"Rask-Andersen H, Stahle J (1980) Immunodefence of the inner ear? Lymphocyte\u2013macrophage interaction in the endolymphatic sac. Acta Oto Laryngol 89(3\u20134):283\u2013294","journal-title":"Acta Oto Laryngol"},{"key":"1091_CR147","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1007\/978-3-7091-6912-4_6","volume":"20","author":"F Resche","year":"1993","unstructured":"Resche F, Moisan JP, Mantoura J et al (1993) Haemangioblastoma, haemangioblastomatosis, and von Hippel\u2013Lindau disease. Adv Tech Stand Neurosurg 20:197\u2013304","journal-title":"Adv Tech Stand Neurosurg"},{"key":"1091_CR148","doi-asserted-by":"crossref","first-page":"1452","DOI":"10.1016\/j.humpath.2006.05.004","volume":"37","author":"CH Rickert","year":"2006","unstructured":"Rickert CH, Hasselblatt M, Jeibmann A, Paulus W (2006) Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles. Hum Pathol 37:1452\u20131457","journal-title":"Hum Pathol"},{"issue":"6","key":"1091_CR149","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1111\/j.1440-1789.2010.01109.x","volume":"30","author":"AL Rivera","year":"2010","unstructured":"Rivera AL, Takei H, Zhai J, Shen SS, Ro JY, Powell SZ (2010) Useful immunohistochemical markers in differentiating hemangioblastoma versus metastatic renal cell carcinoma. Neuropathology 30(6):580\u2013585","journal-title":"Neuropathology"},{"key":"1091_CR150","first-page":"179","volume":"38","author":"G Roussy","year":"1930","unstructured":"Roussy G, Oberling C (1930) Les tumeurs angiomateuses des centres nerveux. Presse Med 38:179\u2013185","journal-title":"Presse Med"},{"issue":"5","key":"1091_CR151","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1007\/s00401-005-0999-3","volume":"109","author":"S Roy","year":"2005","unstructured":"Roy S, Chu A, Trojanowski JQ, Zhang PJ (2005) D2\u201340, a novel monoclonal antibody against the M2A antigen as a marker to distinguish hemangioblastomas from renal cell carcinomas. Acta Neuropathol 109(5):497\u2013502","journal-title":"Acta Neuropathol"},{"key":"1091_CR152","volume-title":"Pathology","author":"E Rubin","year":"1999","unstructured":"Rubin E, Farber JL (1999) Pathology. Lippincott Raven, PA"},{"issue":"2","key":"1091_CR153","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1016\/j.cancergencyto.2010.04.013","volume":"200","author":"IM Schaefer","year":"2010","unstructured":"Schaefer IM, Martinez R, Enders C et al (2010) Molecular cytogenetics of malignant pheochromocytoma with cerebral metastasis. Cancer Genet Cytogenet 200(2):194\u2013197","journal-title":"Cancer Genet Cytogenet"},{"issue":"7","key":"1091_CR154","doi-asserted-by":"crossref","first-page":"884","DOI":"10.1101\/gad.1648608","volume":"22","author":"S Schlisio","year":"2008","unstructured":"Schlisio S, Kenchappa RS, Vredeveld LC et al (2008) The kinesin KIF1Bbeta acts downstream from EglN3 to induce apoptosis and is a potential 1p36 tumor suppressor. Genes Dev 22(7):884\u2013893","journal-title":"Genes Dev"},{"issue":"6161","key":"1091_CR155","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1038\/332268a0","volume":"332","author":"BR Seizinger","year":"1988","unstructured":"Seizinger BR, Rouleau GA, Ozelius LJ et al (1988) Von Hippel\u2013Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma. Nature 332(6161):268\u2013269","journal-title":"Nature"},{"issue":"10","key":"1091_CR156","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1038\/nrc1187","volume":"3","author":"GL Semenza","year":"2003","unstructured":"Semenza GL (2003) Targeting HIF-1 for cancer therapy. Nat Rev Cancer 3(10):721\u2013732","journal-title":"Nat Rev Cancer"},{"key":"1091_CR157","unstructured":"Shively S (2010) Effects of von Hippel\u2013Lindau gene deficiency on the human central nervous system. Institute for Biomedical Sciences. The George Washington University, Washington DC"},{"issue":"4","key":"1091_CR158","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1002\/path.2435","volume":"216","author":"SB Shively","year":"2008","unstructured":"Shively SB, Beltaifa S, Gehrs B et al (2008) Protracted haemangioblastic proliferation and differentiation in von Hippel\u2013Lindau disease. J Pathol 216(4):514\u2013520","journal-title":"J Pathol"},{"issue":"8","key":"1091_CR159","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1038\/modpathol.2011.61","volume":"24","author":"SB Shively","year":"2011","unstructured":"Shively SB, Falke EA, Li J et al (2011) Developmentally arrested structures preceding cerebellar tumors in von Hippel\u2013Lindau disease. Mod Pathol 24(8):1023\u20131030","journal-title":"Mod Pathol"},{"issue":"4","key":"1091_CR160","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1038\/nrm2354","volume":"9","author":"MC Simon","year":"2008","unstructured":"Simon MC, Keith B (2008) The role of oxygen availability in embryonic development and stem cell function. Nat Rev Mol Cell Biol 9(4):285\u2013296","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"1","key":"1091_CR161","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1006\/dbio.1999.9236","volume":"209","author":"AM Sinclair","year":"1999","unstructured":"Sinclair AM, Gottgens B, Barton LM et al (1999) Distinct 5\u2032 SCL enhancers direct transcription to developing brain, spinal cord, and endothelium: neural expression is mediated by GATA factor binding sites. Dev Biol 209(1):128\u2013142","journal-title":"Dev Biol"},{"issue":"2","key":"1091_CR162","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/S0039-6257(01)00245-4","volume":"46","author":"AD Singh","year":"2001","unstructured":"Singh AD, Shields CL, Shields JA (2001) von Hippel\u2013Lindau disease. Surv Ophthalmol 46(2):117\u2013142","journal-title":"Surv Ophthalmol"},{"issue":"3","key":"1091_CR163","doi-asserted-by":"crossref","first-page":"568","DOI":"10.3171\/JNS\/2008\/109\/9\/0568","volume":"109","author":"SM Sorscher","year":"2008","unstructured":"Sorscher SM (2008) Metastasis in von Hippel\u2013Lindau disease. J Neurosurg 109(3):568","journal-title":"J Neurosurg"},{"key":"1091_CR164","doi-asserted-by":"crossref","first-page":"751","DOI":"10.3171\/jns.1960.17.4.0751","volume":"17","author":"AA Stein","year":"1960","unstructured":"Stein AA, Schilp AO, Whitfield RD (1960) The histogenesis of hemangioblastoma of the brain. A review of twenty-one cases. J Neurosurg 17:751\u2013761","journal-title":"J Neurosurg"},{"issue":"36","key":"1091_CR165","doi-asserted-by":"crossref","first-page":"4515","DOI":"10.3748\/wjg.v16.i36.4515","volume":"16","author":"K Tamura","year":"2010","unstructured":"Tamura K, Nishimori I, Ito T, Yamasaki I, Igarashi H, Shuin T (2010) Diagnosis and management of pancreatic neuroendocrine tumor in von Hippel\u2013Lindau disease. World J Gastroenterol 16(36):4515\u20134518","journal-title":"World J Gastroenterol"},{"issue":"9","key":"1091_CR166","doi-asserted-by":"crossref","first-page":"866","DOI":"10.1016\/S0046-8177(84)80148-3","volume":"15","author":"A Tanimura","year":"1984","unstructured":"Tanimura A, Nakamura Y, Hachisuka H, Tanimura Y, Fukumura A (1984) Hemangioblastoma of the central nervous system: nature of the stromal cells as studied by the immunoperoxidase technique. Hum Pathol 15(9):866\u2013869","journal-title":"Hum Pathol"},{"issue":"5","key":"1091_CR167","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1038\/ncb1579","volume":"9","author":"CR Thoma","year":"2007","unstructured":"Thoma CR, Frew IJ, Hoerner CR, Montani M, Moch H, Krek W (2007) pVHL and GSK3beta are components of a primary cilium-maintenance signalling network. Nat Cell Biol 9(5):588\u2013595","journal-title":"Nat Cell Biol"},{"issue":"4","key":"1091_CR168","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1007\/s12022-008-9047-x","volume":"19","author":"AS Tischler","year":"2008","unstructured":"Tischler AS (2008) Pheochromocytoma: time to stamp out \u201cmalignancy\u201d? Endocr Pathol 19(4):207\u2013208","journal-title":"Endocr Pathol"},{"issue":"4","key":"1091_CR169","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1093\/ajcp\/107.4.459","volume":"107","author":"JY Tse","year":"1997","unstructured":"Tse JY, Wong JH, Lo KW, Poon WS, Huang DP, Ng HK (1997) Molecular genetic analysis of the von Hippel\u2013Lindau disease tumor suppressor gene in familial and sporadic cerebellar hemangioblastomas. Am J Clin Pathol 107(4):459\u2013466","journal-title":"Am J Clin Pathol"},{"issue":"2","key":"1091_CR170","doi-asserted-by":"crossref","first-page":"284","DOI":"10.3171\/jns.2005.103.2.0284","volume":"103","author":"TW Vogel","year":"2005","unstructured":"Vogel TW, Vortmeyer AO, Lubensky IA et al (2005) Coexpression of erythropoietin and its receptor in endolymphatic sac tumors. J Neurosurg 103(2):284\u2013288","journal-title":"J Neurosurg"},{"key":"1091_CR171","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1007\/BF01966371","volume":"79","author":"E Hippel von","year":"1911","unstructured":"von Hippel E (1911) Die anatomische Grundlage der von mir beschriebenen sehr seltenen Erkrankung der Netzhaut. Albrecht Von Graefes Arch F Ophth 79:350\u2013377","journal-title":"Albrecht Von Graefes Arch F Ophth"},{"issue":"13","key":"1091_CR172","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1093\/jnci\/89.13.970-a","volume":"89","author":"AO Vortmeyer","year":"1997","unstructured":"Vortmeyer AO, Choo D, Pack SD, Oldfield E, Zhuang Z (1997) von Hippel\u2013Lindau disease gene alterations associated with endolymphatic sac tumor. J Natl Cancer Inst 89(13):970\u2013972","journal-title":"J Natl Cancer Inst"},{"issue":"21","key":"1091_CR173","first-page":"7051","volume":"63","author":"AO Vortmeyer","year":"2003","unstructured":"Vortmeyer AO, Frank S, Jeong SY et al (2003) Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel\u2013Lindau disease. Cancer Res 63(21):7051\u20137055","journal-title":"Cancer Res"},{"issue":"5","key":"1091_CR174","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1016\/S0046-8177(97)90075-7","volume":"28","author":"AO Vortmeyer","year":"1997","unstructured":"Vortmeyer AO, Gnarra JR, Emmert-Buck MR et al (1997) von Hippel\u2013Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel\u2013Lindau disease. Hum Pathol 28(5):540\u2013543","journal-title":"Hum Pathol"},{"issue":"3","key":"1091_CR175","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1002\/path.2062","volume":"210","author":"AO Vortmeyer","year":"2006","unstructured":"Vortmeyer AO, Tran MG, Zeng W et al (2006) Evolution of VHL tumourigenesis in nerve root tissue. J Pathol 210(3):374\u2013382","journal-title":"J Pathol"},{"issue":"11","key":"1091_CR176","doi-asserted-by":"crossref","first-page":"1626","DOI":"10.1212\/01.WNL.0000096168.85260.60","volume":"61","author":"AO Vortmeyer","year":"2003","unstructured":"Vortmeyer AO, Weil RJ, Zhuang Z (2003) Proteomic applications for differential diagnosis of histologically identical tumors. Neurology 61(11):1626\u20131627","journal-title":"Neurology"},{"issue":"5","key":"1091_CR177","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1002\/ana.20090","volume":"55","author":"AO Vortmeyer","year":"2004","unstructured":"Vortmeyer AO, Yuan Q, Lee YS, Zhuang Z, Oldfield EH (2004) Developmental effects of von Hippel\u2013Lindau gene deficiency. Ann Neurol 55(5):721\u2013728","journal-title":"Ann Neurol"},{"issue":"4","key":"1091_CR178","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1007\/BF00463931","volume":"245","author":"PA Wackym","year":"1988","unstructured":"Wackym PA, Glasscock ME 3rd, Linthicum FH Jr, Friberg U, Rask-Andersen H (1988) Immunohistochemical localization of Na+, K+-ATPase in the human endolymphatic sac. Arch Otorhinolaryngol 245(4):221\u2013223","journal-title":"Arch Otorhinolaryngol"},{"issue":"5","key":"1091_CR179","doi-asserted-by":"crossref","first-page":"1475","DOI":"10.1016\/S0022-5347(05)68930-6","volume":"161","author":"MM Walther","year":"1999","unstructured":"Walther MM, Choyke PL, Glenn G et al (1999) Renal cancer in families with hereditary renal cancer: prospective analysis of a tumor size threshold for renal parenchymal sparing surgery. J Urol 161(5):1475\u20131479","journal-title":"J Urol"},{"issue":"6","key":"1091_CR180","doi-asserted-by":"crossref","first-page":"2010","DOI":"10.1016\/S0022-5347(01)66674-6","volume":"154","author":"MM Walther","year":"1995","unstructured":"Walther MM, Lubensky IA, Venzon D, Zbar B, Linehan WM (1995) Prevalence of microscopic lesions in grossly normal renal parenchyma from patients with von Hippel\u2013Lindau disease, sporadic renal cell carcinoma and no renal disease: clinical implications. J Urol 154(6):2010\u20132014","journal-title":"J Urol"},{"issue":"3 Pt 1","key":"1091_CR181","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1097\/00005392-199909010-00004","volume":"162","author":"MM Walther","year":"1999","unstructured":"Walther MM, Reiter R, Keiser HR et al (1999) Clinical and genetic characterization of pheochromocytoma in von Hippel\u2013Lindau families: comparison with sporadic pheochromocytoma gives insight into natural history of pheochromocytoma. J Urol 162(3 Pt 1):659\u2013664","journal-title":"J Urol"},{"issue":"1","key":"1091_CR182","doi-asserted-by":"crossref","first-page":"82","DOI":"10.3171\/jns.2003.98.1.0082","volume":"98","author":"J Wanebo","year":"2003","unstructured":"Wanebo J, Lonser R, Glenn G, Oldfield E (2003) The natural history of hemangioblastomas of the central nervous system in patients with von Hippel\u2013Lindau disease. J Neurosurg 98(1):82\u201394","journal-title":"J Neurosurg"},{"issue":"7","key":"1091_CR183","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1097\/PAS.0b013e3181609d7d","volume":"32","author":"N Weinbreck","year":"2008","unstructured":"Weinbreck N, Marie B, Bressenot A et al (2008) Immunohistochemical markers to distinguish between hemangioblastoma and metastatic clear-cell renal cell carcinoma in the brain: utility of aquaporin1 combined with cytokeratin AE1\/AE3 immunostaining. Am J Surg Pathol 32(7):1051\u20131059","journal-title":"Am J Surg Pathol"},{"issue":"9","key":"1091_CR184","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1007\/s00109-009-0504-x","volume":"87","author":"MS Wiesener","year":"2009","unstructured":"Wiesener MS, Maxwell PH, Eckardt KU (2009) Novel insights into the role of the tumor suppressor von Hippel Lindau in cellular differentiation, ciliary biology, and cyst repression. J Mol Med (Berl) 87(9):871\u2013877","journal-title":"J Mol Med (Berl)"},{"key":"1091_CR185","volume-title":"Gray\u2019s anatomy","author":"PL Williams","year":"1995","unstructured":"Williams PL (1995) Gray\u2019s anatomy. Churchill Livingstone, New York"},{"issue":"9","key":"1091_CR186","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1016\/j.jocn.2009.12.027","volume":"17","author":"J Xiong","year":"2010","unstructured":"Xiong J, Chu SG, Wang Y, Zhu JJ, Li C, Mao Y (2010) Metastasis of renal cell carcinoma to a haemangioblastoma of the medulla oblongata in von Hippel\u2013Lindau syndrome. J Clin Neurosci 17(9):1213\u20131215","journal-title":"J Clin Neurosci"},{"issue":"4","key":"1091_CR187","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1097\/01.mpa.0000240604.26312.e4","volume":"33","author":"I Yamasaki","year":"2006","unstructured":"Yamasaki I, Nishimori I, Ashida S, Kohsaki T, Onishi S, Shuin T (2006) Clinical characteristics of pancreatic neuroendocrine tumors in Japanese patients with von Hippel\u2013Lindau disease. Pancreas 33(4):382\u2013385","journal-title":"Pancreas"},{"issue":"3","key":"1091_CR188","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1038\/ncb1699","volume":"10","author":"AP Young","year":"2008","unstructured":"Young AP, Schlisio S, Minamishima YA et al (2008) VHL loss actuates a HIF-independent senescence programme mediated by Rb and p400. Nat Cell Biol 10(3):361\u2013369","journal-title":"Nat Cell Biol"},{"issue":"1","key":"1091_CR189","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1227\/00006123-199107000-00006","volume":"29","author":"N Zec","year":"1991","unstructured":"Zec N, Cera P, Towfighi J (1991) Extramedullary hematopoiesis in cerebellar hemangioblastoma. Neurosurgery 29(1):34\u201337","journal-title":"Neurosurgery"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-013-1091-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00401-013-1091-z\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-013-1091-z","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,8]],"date-time":"2022-02-08T10:04:23Z","timestamp":1644314663000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00401-013-1091-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,2,12]]},"references-count":189,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["1091"],"URL":"https:\/\/doi.org\/10.1007\/s00401-013-1091-z","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,2,12]]}}}