{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T19:04:17Z","timestamp":1780340657087,"version":"3.54.1"},"reference-count":37,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Introduction: Cerebellopontine angle (CPA) tumors are rare in children. As a result, knowledge on them is still limited, often concerning old series. The goal of this study is to provide an update on these challenging neoplasms by presenting a large series compared with those available in the literature and focusing on tumor characteristics, molecular pattern, extent of tumor removal, surgical complications, and outcome. Methods: All children with CPA tumors consecutively operated on between 2010 and 2020 (minimum follow-up: 5 years) and with complete follow-up data were considered. Retro-sigmoid approach was used for tumors arising from CPA (group A) while a midline sub-occipital was used for those extending into CPA (Group B). Intraoperative neuronavigation, neuro-monitoring, and ultrasounds were routinely utilized. Results: 48 children (54 tumors) were included (mean age at surgery: 6.9 years, 38% infants, M\/F ratio 1.1). Hydrocephalus was present at diagnosis in 27% of cases. Gross total resection of the tumor was obtained in 59% of cases, and subtotal and partial resection in 24% and 17%, respectively. Complications occurred in 25% of cases. Group A was composed of 23 children: the most common tumor was schwannoma (43%) followed by ependymomas, medulloblastoma, AT\/RT (13% each), and less common histotypes. Group B was composed of 25 children: ependymomas (60%), AT\/RT (20%), medulloblastoma (12%), others (8%). All but one ependymomas belonged to PF-A molecular group, while medulloblastomas were equally divided between WNT and Sonic-Hedgehog. The overall survival rate after a mean 7.2-year follow-up is 71%. A total of 14 patients died because of tumor or disease progression. No statistical differences between the two groups were detected as far as demographic data, tumor growing pattern, extent of tumor removal, complication rate, and overall survival were concerned. Only the mean tumor diameter was significantly longer in group B (3.9 cm vs. 3.3 cm). Apart from some differences in the demography, the extent of tumor removal and complications, no relevant differences were noticed among the series analyzed. Conclusions: Pediatric CPA tumors are uncommon but not rare and present significant management challenges. Surgery is demanding. The long-term survival is poorly improved compared with the past and compared with other posterior fossa tumors, the prognosis is mainly related to the biological tumor characteristics and the adjuvant treatments rather than the surgical excision.<\/jats:p>","DOI":"10.3390\/diagnostics16010131","type":"journal-article","created":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T09:18:56Z","timestamp":1767345536000},"page":"131","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Cerebello-Pontine Angle Tumors in Children: An Update on Challenging Neoplasms"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3088-0822","authenticated-orcid":false,"given":"Luca","family":"Massimi","sequence":"first","affiliation":[{"name":"Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"},{"name":"Department of Neuroscience, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliano","family":"Di Monaco","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacopo","family":"Ciccani","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Bianchi","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0071-3126","authenticated-orcid":false,"given":"Paolo","family":"Frassanito","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianpiero","family":"Tamburrini","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"},{"name":"Department of Neuroscience, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,1,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1007\/s11060-013-1067-9","article-title":"Tumors in the Cerebellopontine Angle in Children: Warning of a High Probability of Malignancy","volume":"112","author":"Phi","year":"2013","journal-title":"J. Neurooncol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1097\/MPH.0b013e3181acd842","article-title":"Treatment of Cerebellopontine Angle Tumors in Children: A Single Institution\u2019s Experience","volume":"31","author":"Tsai","year":"2009","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1111\/j.1365-2273.1996.tb01733.x","article-title":"Surgical Results in Unusual Cerebellopontine Angle Tumours","volume":"21","author":"Grey","year":"1996","journal-title":"Clin. Otolaryngol. Allied Sci."},{"key":"ref_4","first-page":"217","article-title":"Nonvestibular Schwannoma Tumors in the Cerebellopontine Angle: A Structured Approach and Management Guidelines","volume":"18","author":"Springborg","year":"2008","journal-title":"Skull Base Off. J. N. Am. Skull Base Soc. Al"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1016\/B978-0-444-53502-3.00013-6","article-title":"Tumors of the Cerebellopontine Angle","volume":"105","author":"Samii","year":"2012","journal-title":"Handb. Clin. Neurol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/s10238-025-01648-5","article-title":"Advancing Medulloblastoma Therapy: Strategies and Survival Insights","volume":"25","author":"Pan","year":"2025","journal-title":"Clin. Exp. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1867","DOI":"10.1002\/cncr.31995","article-title":"Survival and Functional Outcomes of Molecularly Defined Childhood Posterior Fossa Ependymoma: Cure at a Cost","volume":"125","author":"Zapotocky","year":"2019","journal-title":"Cancer"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1301","DOI":"10.3171\/2014.10.JNS141465","article-title":"The Combined Microscopic-Endoscopic Technique for Radical Resection of Cerebellopontine Angle Tumors","volume":"123","author":"Abolfotoh","year":"2015","journal-title":"J. Neurosurg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1007\/s00701-008-0022-6","article-title":"Antibiotic Impregnated External Ventricular Drainage and Third Ventriculostomy in the Management of Hydrocephalus Associated with Posterior Cranial Fossa Tumours","volume":"150","author":"Tamburrini","year":"2008","journal-title":"Acta Neurochir."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e13068","DOI":"10.1111\/bpa.13068","article-title":"Updates in the Classification of Ependymal Neoplasms: The 2021 WHO Classification and Beyond","volume":"32","author":"Kresbach","year":"2022","journal-title":"Brain Pathol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"353","DOI":"10.3171\/2019.5.PEDS18381","article-title":"Medulloblastoma in the Age of Molecular Subgroups: A Review","volume":"24","author":"Juraschka","year":"2019","journal-title":"J. Neurosurg. Pediatr."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1229","DOI":"10.1007\/s00381-009-0886-7","article-title":"Advances in Surgical Techniques for Resection of Childhood Cerebellopontine Angle Ependymomas Are Key to Survival","volume":"25","author":"Sanford","year":"2009","journal-title":"Childs Nerv. Syst."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1007\/s00381-006-0208-2","article-title":"Cerebellopontine Angle Lesions in Children","volume":"23","author":"Sosa","year":"2007","journal-title":"Childs Nerv. Syst."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1739","DOI":"10.1007\/s00381-015-2747-x","article-title":"Cerebellopontine Angle Tumors in Infants and Children","volume":"31","author":"Tomita","year":"2015","journal-title":"Childs Nerv. Syst."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e568","DOI":"10.1016\/j.wneu.2023.09.003","article-title":"Global, Regional, and National Incidence and Mortality Trends in Pediatric Central Nervous System Tumors over the Past 2 Decades","volume":"179","author":"Lu","year":"2023","journal-title":"World Neurosurg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"iii1","DOI":"10.1093\/neuonc\/noab200","article-title":"CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014\u20132018","volume":"23","author":"Ostrom","year":"2021","journal-title":"Neuro-Oncology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1186\/s13023-023-02911-2","article-title":"Incidence and Prevalence of Neurofibromatosis Type 1 and 2: A Systematic Review and Meta-Analysis","volume":"18","author":"Lee","year":"2023","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"49","DOI":"10.3171\/2016.9.JNS161463","article-title":"Gamma Knife Radiosurgery for Treatment of Growing Vestibular Schwannomas in Patients with Neurofibromatosis Type 2: A Matched Cohort Study with Sporadic Vestibular Schwannomas","volume":"128","author":"Kruyt","year":"2018","journal-title":"J. Neurosurg."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1227\/neu.0000000000002039","article-title":"Intraoperative Corticobulbar Motor Evoked Potential in Cerebellopontine Angle Surgery: A Clinically Meaningful Tool to Predict Early and Late Facial Nerve Recovery","volume":"91","author":"Stifano","year":"2022","journal-title":"Neurosurgery"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"864","DOI":"10.1016\/j.clinph.2020.12.025","article-title":"Intraoperative Transcranial Facial Motor Evoked Potential Monitoring in Surgery of Cerebellopontine Angle Tumors Predicts Early and Late Postoperative Facial Nerve Function","volume":"132","author":"Hiruta","year":"2021","journal-title":"Clin. Neurophysiol. Off. J. Int. Fed. Clin. Neurophysiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"182","DOI":"10.25259\/SNI_14_2024","article-title":"Facial Nerve Electrical Motor Evoked Potential in Cerebellopontine Angle Tumors for Its Anatomical and Functional Preservation","volume":"15","author":"Khan","year":"2024","journal-title":"Surg. Neurol. Int."},{"key":"ref_22","first-page":"123","article-title":"Intraoperative Auditory System Monitoring","volume":"81","author":"Miyazaki","year":"2018","journal-title":"Adv. Otorhinolaryngol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"73","DOI":"10.5603\/PJNNS.a2020.0001","article-title":"Reliability of Diffusion Tensor Tractography of Facial Nerve in Cerebello-Pontine Angle Tumours","volume":"54","author":"Szmuda","year":"2020","journal-title":"Neurol. Neurochir. Pol."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Frassanito, P., Stifano, V., Bianchi, F., Tamburrini, G., and Massimi, L. (2023). Enhancing the Reliability of Intraoperative Ultrasound in Pediatric Space-Occupying Brain Lesions. Diagnostics, 13.","DOI":"10.3390\/diagnostics13050971"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1007\/s00381-023-06138-1","article-title":"Exoscopic Microneurosurgery in Pediatric Brain Tumors: An Ideal Tool for Complex and Peculiar Anatomo-Topographic Scenarios?","volume":"40","author":"Trezza","year":"2024","journal-title":"Childs Nerv. Syst."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1097\/MAO.0000000000003347","article-title":"The Role of Endoscopy in the Resection of Sporadic Vestibular Schwannomas: A Systematic Review of Surgical Outcomes","volume":"43","author":"Chiuta","year":"2022","journal-title":"Otol. Neurotol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"578","DOI":"10.4103\/0028-3886.319208","article-title":"Endoscopic-Assisted Microsurgery for Vestibular Schwannomas: Operative Nuances","volume":"69","author":"Shrivastava","year":"2021","journal-title":"Neurol. India"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"5445","DOI":"10.1007\/s00405-025-09427-4","article-title":"A Systematic Review on the Role of the Endoscope in the Surgical Management of Cerebellopontine Angle Tumors: Is It Time to Draw the Conclusion?","volume":"282","author":"Froelich","year":"2025","journal-title":"Eur. Arch. Oto-Rhino-Laryngol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"59","DOI":"10.3171\/2022.10.JNS22489","article-title":"From White to Blue Light: Evolution of Endoscope-Assisted Intracranial Tumor Neurosurgery and Expansion to Intraaxial Tumors","volume":"139","author":"Ruzevick","year":"2023","journal-title":"J. Neurosurg."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1080\/02688697.2020.1867058","article-title":"Surgical Management of Cerebellopontine Angle Epidermoid Cysts: An Institutional Experience of 10 Years","volume":"36","author":"Vernon","year":"2022","journal-title":"Br. J. Neurosurg."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1007\/s10143-025-03938-8","article-title":"Fully Endoscopic Approach to Cerebellopontine Angle Tumors: A Systematic Review and Meta-Analysis","volume":"49","author":"Gomes","year":"2025","journal-title":"Neurosurg. Rev."},{"key":"ref_32","first-page":"458","article-title":"General Overview of Posterior Fossa Tumors and Surgical Treatment","volume":"18","author":"Caldarelli","year":"1993","journal-title":"Rays"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1007\/BF01476280","article-title":"Intracranial Tumours in the First Year of Life. A Report on 51 Cases","volume":"123","author":"Ceddia","year":"1993","journal-title":"Acta Neurochir."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1303","DOI":"10.1007\/s00381-009-0874-y","article-title":"Survival Following Treatment for Intracranial Ependymoma: A Review","volume":"25","author":"Tamburrini","year":"2009","journal-title":"Childs Nerv. Syst."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e504","DOI":"10.1016\/j.wneu.2023.10.089","article-title":"Management of Atypical Teratoid\/Rhabdoid Tumors in the Pediatric Population: A Systematic Review and Meta-Analysis","volume":"181","author":"Gupta","year":"2024","journal-title":"World Neurosurg."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/bs.acr.2025.08.007","article-title":"Current Advances in the Management of Atypical Teratoid Rhabdoid Tumors (ATRT)","volume":"167","author":"Huang","year":"2025","journal-title":"Adv. Cancer Res."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/S1470-2045(15)00581-1","article-title":"Prognostic Value of Medulloblastoma Extent of Resection after Accounting for Molecular Subgroup: A Retrospective Integrated Clinical and Molecular Analysis","volume":"17","author":"Thompson","year":"2016","journal-title":"Lancet Oncol."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/16\/1\/131\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T09:33:34Z","timestamp":1767346414000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/16\/1\/131"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,1]]},"references-count":37,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1]]}},"alternative-id":["diagnostics16010131"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics16010131","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,1]]}}}