{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T13:14:27Z","timestamp":1791897267255,"version":"4.3.4"},"reference-count":19,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2006,8]]},"abstract":"\n Object<\/jats:title>\n In the course of their lives most patients with von Hippel\u2013Lindau (VHL) disease require treatment for several symptom-producing hemangioblastomas of the cerebellum, brainstem, or spinal cord. However, many tumors never produce symptoms and do not require treatment. Detection at an early stage of lesions that will later produce symptoms and ultimately require treatment would allow for earlier excision of hemangioblastomas of the spinal cord, brainstem, or cerebellum, and may identify cerebellar hemangioblastomas that can be treated with radiosurgery at a stage before treatment is contraindicated because of tumor size or the presence of an associated cyst.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n To identify features predictive of symptom development that might allow for earlier treatment of smaller, presymptomatic hemangioblastomas in patients with VHL disease, the authors reviewed and analyzed the serial clinical and imaging findings in all patients with VHL disease who were followed up at the National Institutes of Health for more than 10 years. Features predictive of symptom formation were determined by recursive partition and regression analyses.<\/jats:p>\n \n Nineteen patients (10 men and nine women; mean age 32.6 \u00b1 11.6 years) harboring a total of 143 hemangioblastomas were identified (mean follow-up duration 12.4 \u00b1 1.4 years). Hemangioblastomas were located in the cerebellum (68 hemangioblastomas, 48% of patients), brainstem (17 hemangioblastomas, 12% of patients), and spinal cord (58 hemangioblastomas, 40% of patients). Despite measurable growth in almost all hemangioblastomas (138 lesions, 97% of patients), only 58 (41% of patients) became symptomatic. Hemangioblastomas grew in a stuttering pattern. (mean growth period 13 \u00b1 15 months, mean quiescent period 25 \u00b1 19 months). Twenty-six (45%) of the hemangioblastomas that eventually produced symptoms were not among the tumors that were apparent on the initial MR imaging study. Depending on location, the hemangioblastoma size and\/or tumor and cyst growth rates predicted symptom development and the need for treatment (p < 0.05). Cerebellar hemangioblastomas growing faster than 112 mm\n 3<\/jats:sup>\n \/ month or larger than 69 mm\n 3<\/jats:sup>\n with associated tumor and cyst growth rates greater than 14 mm\n 3<\/jats:sup>\n \/month became symptomatic (100% sensitivity, 72% specificity). Brainstem hemangioblastomas larger than 245 mm\n 3<\/jats:sup>\n with growth rates greater than 0.1 mm\n 3<\/jats:sup>\n \/month became symptomatic (75% sensitivity, 89% specificity). Spinal hemangioblastomas larger than 22 mm\n 3<\/jats:sup>\n became symptomatic (79% sensitivity, 94% specificity).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n Because hemangioblastomas exhibit a stuttering growth pattern, frequently remain asymptomatic, and do not require treatment for long intervals, unqualified radiographic progression is not an indication for treatment. Basing the decision to intervene in individual tumors solely on radiographic progression would have resulted in approximately four additional procedures per patient during the 10-year study period. Threshold values are presented for tumor size and\/or tumor and cyst growth rates that can be used to predict symptom formation and future need for treatment.<\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/jns.2006.105.2.248","type":"journal-article","created":{"date-parts":[[2008,1,24]],"date-time":"2008-01-24T07:37:17Z","timestamp":1201160237000},"page":"248-255","source":"Crossref","is-referenced-by-count":165,"title":["Long-term natural history of hemangioblastomas in patients with von Hippel\u2013Lindau disease: implications for treatment"],"prefix":"10.3171","volume":"105","author":[{"given":"Joshua M.","family":"Ammerman","sequence":"first","affiliation":[{"name":"Surgical Neurology Branch and Biostatistics Branch, National Institute of Neurological Disorders and Stroke, and Diagnostic Radiology Department, Warren G. Magnusson Clinical Center, National Institutes of Health, Bethesda, Maryland; and Department of Neurological Surgery, George Washington University Medical Center, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell R.","family":"Lonser","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and Biostatistics Branch, National Institute of Neurological Disorders and Stroke, and Diagnostic Radiology Department, Warren G. Magnusson Clinical Center, National Institutes of Health, Bethesda, Maryland; and Department of Neurological Surgery, George Washington University Medical Center, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Dambrosia","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and Biostatistics Branch, National Institute of Neurological Disorders and Stroke, and Diagnostic Radiology Department, Warren G. Magnusson Clinical Center, National Institutes of Health, Bethesda, Maryland; and Department of Neurological Surgery, George Washington University Medical Center, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Butman","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and Biostatistics Branch, National Institute of Neurological Disorders and Stroke, and Diagnostic Radiology Department, Warren G. Magnusson Clinical Center, National Institutes of Health, Bethesda, Maryland; and Department of Neurological Surgery, George Washington University Medical Center, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward H.","family":"Oldfield","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and Biostatistics Branch, National Institute of Neurological Disorders and Stroke, and Diagnostic Radiology Department, Warren G. Magnusson Clinical Center, National Institutes of Health, Bethesda, Maryland; and Department of Neurological Surgery, George Washington University Medical Center, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-1050248","series-title":"Ophthalmology","doi-asserted-by":"publisher","first-page":"1745","DOI":"10.1016\/S0161-6420(02)01159-4","article-title":"Rapid and durable recovery of visual function in a patient with von Hippel-Lindau syndrome after systemic therapy with vascular endothelial growth factor receptor inhibitor su5416","volume":"109","year":"2002"},{"key":"b2-1050248","series-title":"Br J Neurosurg","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1080\/02688699746447","article-title":"Stereotactic multiple arc radiotherapy. IV-haemangioblastoma","volume":"11","year":"1997"},{"key":"b3-1050248","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1097\/00006123-199807000-00018","article-title":"Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery","volume":"43","year":"1998"},{"key":"b4-1050248","series-title":"Neurosurgery","first-page":"55","article-title":"Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease","volume":"48","year":"2001"},{"key":"b5-1050248","series-title":"Am J Ophthalmol","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1016\/S0002-9394(03)00101-6","article-title":"Treatment of von Hippel-Lindau retinal hemangioblastoma by the vascular endothelial growth factor receptor inhibitor SU5416 is more effective for associated macular edema than for hemangioblastomas","volume":"136","year":"2003"},{"key":"b6-1050248","series-title":"Chin Med J","first-page":"552","article-title":"Genetic study of a large Chinese kindred with von Hippel-Lindau disease","volume":"117","year":"2004"},{"key":"b7-1050248","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1007\/s007010070107","article-title":"Stereotactic radiosurgery for hemangioblastomas of the brain","volume":"142","year":"2000"},{"key":"b8-1050248","series-title":"Stereotact Funct Neurosurg","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1159\/000056437","article-title":"Brain tumors with cysts treated with Gamma Knife radiosurgery: is microsurgery indicated?","volume":"72","year":"1999"},{"key":"b9-1050248","series-title":"Lancet","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.1016\/S0140-6736(03)13643-4","article-title":"von Hippel-Lindau disease","volume":"361","year":"2003"},{"key":"b10-1050248","series-title":"J Comput Assist Tomogr","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1097\/00004728-199207000-00004","article-title":"Volume of pituitary macroadenomas: assessment by MRI","volume":"16","year":"1992"},{"key":"b11-1050248","series-title":"JAMA","doi-asserted-by":"publisher","first-page":"943","DOI":"10.1001\/jama.291.8.943","article-title":"Antiangiogenic therapy for von Hippel-Lindau disease","volume":"291","year":"2004"},{"key":"b12-1050248","series-title":"J Med Genet","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1136\/jmg.28.7.443","article-title":"Von Hippel-Lindau disease: a genetic study","volume":"28","year":"1991"},{"key":"b13-1050248","series-title":"Q J Med","doi-asserted-by":"publisher","first-page":"1151","DOI":"10.1093\/qjmed\/77.2.1151","article-title":"Clinical features and natural history of von Hippel-Lindau disease","volume":"77","year":"1990"},{"key":"b14-1050248","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"523","DOI":"10.3171\/jns.1990.72.4.0523","article-title":"Intramedullary ependymoma of the spinal cord","volume":"72","year":"1990"},{"key":"b15-1050248","series-title":"Stereotact Funct Neurosurg","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1159\/000056420","article-title":"Gamma knife radiosurgery for hemangioblastomas","volume":"70","year":"1998"},{"key":"b16-1050248","series-title":"Int J Radiat Oncol Biol Phys","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1016\/S0360-3016(96)80011-3","article-title":"Radiosurgery for hemangioblastoma: results of a multiinstitutional experience","volume":"35","year":"1996"},{"key":"b17-1050248","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"553","DOI":"10.1002\/1097-0142(19820201)49:3<553::AID-CNCR2820490326>3.0.CO;2-A","article-title":"Cerebellar hemangioblastomas","volume":"49","year":"1982"},{"key":"b18-1050248","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"1306","DOI":"10.1227\/01.NEU.0000093497.81390.29","article-title":"Treatment of intramedullary hemangioblastomas, with special attention to von Hippel-Lindau disease","volume":"53","year":"2003"},{"key":"b19-1050248","series-title":"J Neurosurg","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-Lindau disease","volume":"98","year":"2003"}],"container-title":["Journal of Neurosurgery"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/105\/2\/article-p248.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/105\/2\/article-p248.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/105\/2\/article-p248.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,9]],"date-time":"2024-03-09T04:46:26Z","timestamp":1709959586000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/105\/2\/article-p248.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,8]]},"references-count":19,"journal-issue":{"issue":"2"},"URL":"https:\/\/doi.org\/10.3171\/jns.2006.105.2.248","relation":{},"ISSN":["0022-3085"],"issn-type":[{"value":"0022-3085","type":"print"}],"subject":[],"published":{"date-parts":[[2006,8]]}}}