{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T17:14:04Z","timestamp":1794071644918,"version":"4.8.0"},"reference-count":38,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2012,7]]},"abstract":"\n Object<\/jats:title>\n Neurofibromatosis Type 2 (NF2) is a heritable tumor predisposition syndrome that leads to the development of multiple intracranial tumors, including meningiomas and schwannomas. Because the natural history of these tumors has not been determined, their optimal management has not been established. To define the natural history of NF2-associated intracranial tumors and to optimize management strategies, the authors evaluated long-term clinical and radiographic data in patients with NF2.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n Consecutive NF2 patients with a minimum of 4 years of serial clinical and MRI follow-up were analyzed.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n Seventeen patients, 9 males and 8 females, were included in this analysis (mean follow-up 9.5 \u00b1 4.8 years, range 4.0\u201320.7 years). The mean age at initial evaluation was 33.2 \u00b1 15.5 years (range 12.3\u201357.6 years). Patients harbored 182 intracranial neoplasms, 164 of which were assessable for growth rate analysis (18 vestibular schwannomas [VSs], 11 nonvestibular cranial nerve [CN] schwannomas, and 135 meningiomas) and 152 of which were assessable for growth pattern analysis (15 VSs, 9 nonvestibular CN schwannomas, and 128 meningiomas). New tumors developed in patients over the course of the imaging follow-up: 66 meningiomas, 2 VSs, and 2 nonvestibular CN schwannomas. Overall, 45 tumors (29.6%) exhibited linear growth, 17 tumors (11.2%) exhibited exponential growth, and 90 tumors (59.2%) displayed a saltatory growth pattern characterized by alternating periods of growth and quiescence (mean quiescent period 2.3 \u00b1 2.1 years, range 0.4\u201311.7 years). Further, the saltatory pattern was the most frequently identified growth pattern for each tumor type: meningiomas 60.9%, VSs 46.7%, and nonvestibular schwannoma 55.6%. A younger age at the onset of NF2-related symptoms (p = 0.01) and female sex (p = 0.05) were associated with an increased growth rate in meningiomas. The identification of saltatory growth in meningiomas increased with the duration of follow-up (p = 0.01).<\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n Neurofibromatosis Type 2\u2013associated intracranial tumors most frequently demonstrated a saltatory growth pattern. Because new tumors can develop in NF2 patients over their lifetime and because radiographic progression and symptom formation are unpredictable, resection may be best reserved for symptom-producing tumors. Moreover, establishing the efficacy of nonsurgical therapeutic interventions must be based on long-term follow-up (several years).<\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/2012.3.jns111649","type":"journal-article","created":{"date-parts":[[2012,4,13]],"date-time":"2012-04-13T09:49:18Z","timestamp":1334310558000},"page":"109-117","source":"Crossref","is-referenced-by-count":110,"title":["Long-term natural history of neurofibromatosis Type 2\u2013associated intracranial tumors"],"prefix":"10.3171","volume":"117","author":[{"given":"Michael S.","family":"Dirks","sequence":"first","affiliation":[{"name":"Surgical Neurology Branch and"},{"name":"Department of Neurological Surgery, Walter Reed Army Medical Center, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Butman","sequence":"additional","affiliation":[{"name":"Diagnostic Radiology Department, Clinical Center of the National Institutes of Health;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Jeffrey","family":"Kim","sequence":"additional","affiliation":[{"name":"Head and Neck Surgery Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tianxia","family":"Wu","sequence":"additional","affiliation":[{"name":"Clinical Neurosciences Program, National Institute of Neurological Disorders and Stroke;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keaton","family":"Morgan","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne P.","family":"Tran","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell R.","family":"Lonser","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashok R.","family":"Asthagiri","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-jns111649","series-title":"Laryngoscope","first-page":"694","article-title":"Growth rate characteristics of acoustic neuromas associated with neurofibromatosis type 2","volume":"106","author":"Abaza","year":"1996"},{"key":"b2-jns111649","series-title":"J Neurosurg","first-page":"248","article-title":"Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment","volume":"105","author":"Ammerman","year":"2006"},{"key":"b3-jns111649","series-title":"Neurooncol","first-page":"80","article-title":"Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease","volume":"12","author":"Asthagiri","year":"2010"},{"key":"b4-jns111649","series-title":"Lancet","first-page":"1974","article-title":"Neurofibromatosis type 2","volume":"373","author":"Asthagiri","year":"2009"},{"key":"b5-jns111649","series-title":"Am J Hum Genet","first-page":"715","article-title":"Predictors of the risk of mortality in neurofibromatosis 2","volume":"71","author":"Baser","year":"2002"},{"key":"b6-jns111649","series-title":"Neurology","first-page":"1759","article-title":"Evaluation of clinical diagnostic criteria for neurofibromatosis 2","volume":"59","author":"Baser","year":"2002"},{"key":"b7-jns111649","series-title":"J Neurosurg","first-page":"217","article-title":"Predictors of vestibular schwannoma growth in patients with neurofibromatosis Type 2","volume":"96","author":"Baser","year":"2002"},{"key":"b8-jns111649","series-title":"J Med Genet","first-page":"903","article-title":"Methodological issues in longitudinal studies: vestibular schwannoma growth rates in neurofibromatosis 2","volume":"42","author":"Baser","year":"2005"},{"key":"b9-jns111649","series-title":"Acta Neurochir (Wien)","first-page":"42","article-title":"Sex steroid receptors in human meningiomas","volume":"79","author":"Blaauw","year":"1986"},{"key":"b10-jns111649","series-title":"Clin Cancer Res","first-page":"5032","article-title":"Consensus recommendations to accelerate clinical trials for neurofibromatosis type 2","volume":"15","author":"Evans","year":"2009"},{"key":"b11-jns111649","series-title":"Q J Med","first-page":"603","article-title":"A clinical study of type 2 neurofibromatosis","volume":"84","author":"Evans","year":"1992"},{"key":"b12-jns111649","series-title":"Otol Neurotol","first-page":"93","article-title":"Incidence of vestibular schwannoma and neurofibromatosis 2 in the North West of England over a 10-year period: higher incidence than previously thought","volume":"26","author":"Evans","year":"2005"},{"key":"b13-jns111649","series-title":"Otol Neurotol","first-page":"1083","article-title":"Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2","volume":"28","author":"Fisher","year":"2007"},{"key":"b14-jns111649","series-title":"Neurology","first-page":"1766","article-title":"Mononeuropathy multiplex as the initial manifestation of neurofibromatosis type 2","volume":"56","author":"Gijtenbeek","year":"2001"},{"key":"b15-jns111649","series-title":"Acta Neuropathol","first-page":"179","article-title":"Polyneuropathy in neurofibromatosis 2: clinical findings, molecular genetics and neuropathological alterations in sural nerve biopsy specimens","volume":"104","author":"Hagel","year":"2002"},{"key":"b16-jns111649","series-title":"Neurosurgery","first-page":"1314","article-title":"Three-dimensional volumetrics for tracking vestibular schwannoma growth in neurofibromatosis type II","volume":"62","author":"Harris","year":"2008"},{"key":"b17-jns111649","series-title":"J Neurosurg","first-page":"675","article-title":"Serial volumetric assessment of the natural history and growth pattern of incidentally discovered meningiomas. 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