{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T09:18:48Z","timestamp":1774084728194,"version":"3.50.1"},"reference-count":20,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2003,9]]},"abstract":"<jats:p content-type=\"fine-print\"><jats:italic>Object.<\/jats:italic> Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2.<\/jats:p>\n<jats:p content-type=\"fine-print\"><jats:italic>Methods.<\/jats:italic> From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2\u2013287 months). Results of a Kaplan\u2014Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (&lt; 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival.<\/jats:p>\n<jats:p content-type=\"fine-print\"><jats:italic>Conclusions.<\/jats:italic> This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.<\/jats:p>","DOI":"10.3171\/jns.2003.99.3.0480","type":"journal-article","created":{"date-parts":[[2009,5,13]],"date-time":"2009-05-13T17:51:10Z","timestamp":1242237070000},"page":"480-483","source":"Crossref","is-referenced-by-count":83,"title":["Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2"],"prefix":"10.3171","volume":"99","author":[{"given":"Goro","family":"Otsuka","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyoshi","family":"Saito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuya","family":"Nagatani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Yoshida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Acta Neurochir","first-page":"40","article-title":"The comparison between the growth fraction of bilateral vestibular schwannomas in neurofibromatosis 2 (NF2) and unilateral vestibular schwannomas using the monoclonal antibody MIB 1.","volume":"134","author":"Aguiar","year":"1995"},{"key":"b2","series-title":"Arch Neurol","first-page":"201","article-title":"National Institutes of Health Consensus Development Conference Statement on Acoustic Neuroma, December 11\u201313, 1991. 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Prevalence, mutation rate, fitness, and confirmation of maternal transmission effect on severity.","volume":"29","author":"Evans","year":"1992"},{"key":"b8","series-title":"J Med Genet","first-page":"847","article-title":"A genetic study of type 2 neurofibromatosis in the United Kingdom. II. Guidelines for genetic counselling.","volume":"29","author":"Evans","year":"1992"},{"key":"b9","series-title":"JAMA","first-page":"51","article-title":"The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2.","volume":"278","author":"Gutmann","year":"1997"},{"key":"b10","series-title":"Ann Neurol","first-page":"440","article-title":"A point mutation associated with a severe phenotype of neurofibromatosis 2.","volume":"40","author":"MacCollin","year":"1996"},{"key":"b11","series-title":"Neurosurgery","first-page":"1","article-title":"Bilateral acoustic neuromas: clinical aspects, pathogenesis, and treatment.","volume":"10","author":"Martuza","year":"1982"},{"key":"b12","series-title":"Ann Intern Med","first-page":"39","article-title":"NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update.","volume":"113","author":"Mulvihill","year":"1990"},{"key":"b13","series-title":"Am J Med Genet","first-page":"450","article-title":"Neurofibromatosis 2 (NF2): clinical characteristics of 63 affected individuals and clinical evidence for heterogeneity.","volume":"52","author":"Parry","year":"1994"},{"key":"b14","series-title":"Am J Hum Genet","first-page":"529","article-title":"Germ-line mutations in neurofibromatosis 2 gene: correlations with disease severity and retinal abnormalities.","volume":"59","author":"Parry","year":"1996"},{"key":"b15","series-title":"Nature","first-page":"515","article-title":"Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2.","volume":"363","author":"Rouleau","year":"1993"},{"key":"b16","series-title":"Am J Hum Genet","first-page":"331","article-title":"Type of mutation in the neurofibromatosis type 2 gene (NF2) frequently determines severity of disease.","volume":"59","author":"Ruttledge","year":"1996"},{"key":"b17","series-title":"Neurosurgery","first-page":"696","article-title":"Management of vestibular schwannomas (acoustic neuromas): auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2.","volume":"40","author":"Samii","year":"1997"},{"key":"b18","series-title":"J Neurosurg","first-page":"815","article-title":"Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2.","volume":"90","author":"Subach","year":"1999"},{"key":"b19","series-title":"Cell","first-page":"791","article-title":"A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor.","volume":"72","author":"Trofatter","year":"1993"},{"key":"b20","series-title":"Tuberous Sclerosis and Neurofibromatosis: Epidemiology, Pathophysiology, Biology and Management.","first-page":"315","article-title":"A statistical study of central neurofibromatosis in Japan","author":"Yoshida","year":"1990"}],"container-title":["Journal of Neurosurgery"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/99\/3\/article-p480.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/99\/3\/article-p480.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/99\/3\/article-p480.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,3,19]],"date-time":"2023-03-19T23:35:12Z","timestamp":1679268912000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/99\/3\/article-p480.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,9]]},"references-count":20,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3171\/jns.2003.99.3.0480","relation":{},"ISSN":["0022-3085"],"issn-type":[{"value":"0022-3085","type":"print"}],"subject":[],"published":{"date-parts":[[2003,9]]}}}