{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T23:49:06Z","timestamp":1791589746024,"version":"4.3.4"},"reference-count":23,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2013,10]]},"abstract":"<jats:sec>\n                    <jats:title>Object<\/jats:title>\n                    <jats:p>The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age \u2264 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9\u201318.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon.<\/jats:p>\n                    <jats:p>Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2013.6.peds1383","type":"journal-article","created":{"date-parts":[[2013,8,2]],"date-time":"2013-08-02T10:01:40Z","timestamp":1375437700000},"page":"317-324","source":"Crossref","is-referenced-by-count":37,"title":["Pediatric cerebellopontine angle and internal auditory canal tumors"],"prefix":"10.3171","volume":"12","author":[{"given":"Michelle A.","family":"Holman","sequence":"first","affiliation":[{"name":"Departments of Otolaryngology-Head and Neck Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William R.","family":"Schmitt","sequence":"additional","affiliation":[{"name":"Departments of Otolaryngology-Head and Neck Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew L.","family":"Carlson","sequence":"additional","affiliation":[{"name":"Departments of Otolaryngology-Head and Neck Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin L. W.","family":"Driscoll","sequence":"additional","affiliation":[{"name":"Departments of Otolaryngology-Head and Neck Surgery and"},{"name":"Neurologic Surgery, Mayo Clinic School of Medicine, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles W.","family":"Beatty","sequence":"additional","affiliation":[{"name":"Departments of Otolaryngology-Head and Neck Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J.","family":"Link","sequence":"additional","affiliation":[{"name":"Departments of Otolaryngology-Head and Neck Surgery and"},{"name":"Neurologic Surgery, Mayo Clinic School of Medicine, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-peds1383","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"1759","DOI":"10.1212\/01.WNL.0000035638.74084.F4","article-title":"Evaluation of clinical diagnostic criteria for neurofibromatosis 2","volume":"59","author":"Baser","year":"2002"},{"key":"b2-peds1383","series-title":"Otolaryngol Head Neck Surg (1979)","doi-asserted-by":"publisher","first-page":"555","DOI":"10.1177\/019459988008800508","article-title":"Rare tumors of the cerebellopontine angle","volume":"88","author":"Brackmann","year":"1980"},{"key":"b3-peds1383","series-title":"Otolaryngol Head Neck Surg","first-page":"179","article-title":"Committee on Hearing and Equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma). American Academy of Otolaryngology-Head and Neck Surgery Foundation, INC","volume":"113","year":"1995"},{"key":"b4-peds1383","series-title":"Otol Neurotol","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1097\/00129492-200503000-00017","article-title":"Neurotologic skull base surgery in pediatric patients","volume":"26","author":"Cunningham","year":"2005"},{"key":"b5-peds1383","series-title":"Br J Neurosurg","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1080\/02688690500081206","article-title":"Management of the patient and family with neurofibromatosis 2: a consensus conference statement","volume":"19","author":"Evans","year":"2005"},{"key":"b6-peds1383","series-title":"Arch Dis Child","doi-asserted-by":"publisher","first-page":"496","DOI":"10.1136\/adc.81.6.496","article-title":"Paediatric presentation of type 2 neurofibromatosis","volume":"81","author":"Evans","year":"1999"},{"key":"b7-peds1383","series-title":"Q J Med","first-page":"603","article-title":"A clinical study of type 2 neurofibromatosis","volume":"84","author":"Evans","year":"1992"},{"key":"b8-peds1383","series-title":"Otol Neurotol","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1097\/00129492-200501000-00016","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":"b9-peds1383","series-title":"Clin Genet","doi-asserted-by":"publisher","first-page":"354","DOI":"10.1111\/j.1399-0004.2007.00778.x","article-title":"Should NF2 mutation screening be undertaken in patients with an apparently isolated vestibular schwannoma?","volume":"71","author":"Evans","year":"2007"},{"key":"b10-peds1383","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"92","DOI":"10.3171\/JNS\/2008\/108\/01\/0092","article-title":"What are the implications in individuals with unilateral vestibular schwannoma and other neurogenic tumors?","volume":"108","author":"Evans","year":"2008"},{"key":"b11-peds1383","series-title":"Clin Genet","doi-asserted-by":"publisher","first-page":"416","DOI":"10.1111\/j.1399-0004.2011.01816.x","article-title":"Genetic testing and screening of individuals at risk of NF2","volume":"82","author":"Evans","year":"2012"},{"key":"b12-peds1383","series-title":"Arch Otolaryngol","doi-asserted-by":"publisher","first-page":"712","DOI":"10.1001\/archotol.1968.00770010714020","article-title":"Other tumors of the cerebellopontine angle","volume":"88","author":"Hitselberger","year":"1968"},{"key":"b13-peds1383","series-title":"Otolaryngol Head Neck Surg","doi-asserted-by":"publisher","first-page":"146","DOI":"10.1177\/019459988509300202","article-title":"Facial nerve grading system","volume":"93","author":"House","year":"1985"},{"key":"b14-peds1383","series-title":"Otolaryngol Clin North Am","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1016\/S0030-6665(20)30970-1","article-title":"Meningiomas, epidermoids, and other nonacoustic tumors of the cerebellopontine angle","volume":"25","author":"Lalwani","year":"1992"},{"key":"b15-peds1383","series-title":"N Engl J Med","first-page":"684","article-title":"Neurofibromatosis 2 (bilateral acoustic neurofibromatosis)","volume":"318","author":"Martuza","year":"1988"},{"key":"b16-peds1383","series-title":"Clin Oncol (R Coll Radiol)","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1016\/S0936-6555(05)80632-6","article-title":"Rare tumours of the cerebellopontine angle","volume":"7","author":"Moffat","year":"1995"},{"key":"b17-peds1383","series-title":"J Otolaryngol","first-page":"248","article-title":"Other cerebellopontine angle (non-acoustic neuroma) tumors","volume":"11","author":"Nedzelski","year":"1982"},{"key":"b18-peds1383","series-title":"J Child Neurol","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1177\/08830738030180101301","article-title":"Neurofibromatosis 2 in the pediatric population","volume":"18","author":"Nunes","year":"2003"},{"key":"b19-peds1383","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"895","DOI":"10.1056\/NEJM197211022871802","article-title":"Evaluation and surgical treatment of acoustic neuroma","volume":"287","author":"Ojemann","year":"1972"},{"key":"b20-peds1383","series-title":"Otol Neurotol","doi-asserted-by":"publisher","first-page":"863","DOI":"10.1097\/MAO.0b013e318255dd59","article-title":"Prospective evaluation of the clinical profile and referral pattern differences of vestibular schwannomas and other cerebellopontine angle tumors","volume":"33","author":"Ragab","year":"2012"},{"key":"b21-peds1383","series-title":"Laryngoscope","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1097\/00005537-200503000-00011","article-title":"Conservative management of acoustic neuroma: a meta-analysis and proposed treatment algorithm","volume":"115","author":"Smouha","year":"2005"},{"key":"b22-peds1383","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"125","DOI":"10.3171\/2009.3.PEDS08434","article-title":"Sporadic unilateral vestibular schwannoma in the pediatric population. Clinical article","volume":"4","author":"Walcott","year":"2009"},{"key":"b23-peds1383","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1007\/s00381-006-0208-2","article-title":"Cerebellopontine angle lesions in children","volume":"23","author":"Z\u00faccaro","year":"2007"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/12\/4\/article-p317.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/12\/4\/article-p317.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/12\/4\/article-p317.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T08:46:41Z","timestamp":1681548401000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/12\/4\/article-p317.xml"}},"subtitle":["Clinical article"],"short-title":[],"issued":{"date-parts":[[2013,10]]},"references-count":23,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.3171\/2013.6.peds1383","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,10]]}}}