{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T18:31:13Z","timestamp":1791829873826,"version":"4.3.4"},"reference-count":104,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,3,1]]},"abstract":"<jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas commonly associated with neurofibromatosis type 1 (NF1), whose occurrence in schwannomatosis remains poorly understood. This study aimed to characterize MPNSTs in NF type 2 (NF2)\u2013 and SMARCB1-related schwannomatosis through a systematic review and meta-analysis of survival outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>A comprehensive search of the PubMed, Embase, Scopus, SEER (Surveillance, Epidemiology, and End Results), and Web of Science databases was conducted from database inception through January 2024. Clinical, radiological, histopathological, and treatment data were extracted. Primary endpoints included overall survival (OS) and progression-free survival (PFS). Predictors of outcomes were analyzed using Kaplan-Meier survival curves and Cox proportional hazards models.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>A total of 39 cases were identified. NF2-related disease accounted for 67% of cases, while 28% were SMARCB1 related. The mean age at MPNST diagnosis was 33 years (range 9\u201379 years). Pain (41%) and weakness (26%) were the most common presenting symptoms. The mortality rate was 72.9%. No tumors were associated with LZTR1 mutations. Tumor locations varied widely, with involvement of the pelvis, thigh, skull base, forearm\/hand, and cranial nerves. Trends suggested improved survival in the absence of S100 loss, gross-total resection, and a known schwannoma precursor. Conversely, having an intracranial or intraspinal lesion or having NF2 in the setting of prior radiation therapy were found to be associated with decreased survival and increased progression. A risk stratification tool predicted OS (HR 28.0, p &lt; 0.0001) and PFS (HR 12.1, p &lt; 0.0001).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>MPNSTs, although rare in schwannomatosis, can arise even in the absence of prior radiation exposure and may mimic benign schwannomas. A preliminary risk stratification tool may aid in identifying high-risk patients and optimizing treatment approaches, although validation is needed. Given the aggressive nature of MPNSTs and their potential for delayed diagnosis due to their rarity, vigilant monitoring and individualized treatment strategies are crucial. Future research should focus on refining risk prediction models and exploring targeted therapies for schwannomatosis-associated MPNSTs to improve patient outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2025.6.jns251508","type":"journal-article","created":{"date-parts":[[2025,11,15]],"date-time":"2025-11-15T01:40:49Z","timestamp":1763170849000},"page":"642-652","source":"Crossref","is-referenced-by-count":2,"title":["Malignant peripheral nerve sheath tumors in schwannomatosis: systematic review and meta-analysis"],"prefix":"10.3171","volume":"144","author":[{"given":"Ken","family":"Porche","sequence":"first","affiliation":[{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirsten M.","family":"Hayford","sequence":"additional","affiliation":[{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chloe","family":"Gui","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, University Health Network, University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suganth","family":"Suppiah","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, University Health Network, University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Spinner","sequence":"additional","affiliation":[{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Central Nervous System Tumours. WHO Classification of Tumours, 5th Edition, Volume 6","year":"2021","unstructured":"Central Nervous System Tumours. WHO Classification of Tumours, 5th Edition, Volume 6. World Health Organization; 2021."},{"issue":"1","key":"b2","series-title":"Neurosurgery","first-page":"38","article-title":"Malignant peripheral nerve sheath tumors are not a feature of neurofibromatosis type 2 in the unirradiated patient","volume":"83","author":"King AT","year":"2018","unstructured":"King AT, Rutherford SA, Hammerbeck-Ward C,  Malignant peripheral nerve sheath tumors are not a feature of neurofibromatosis type 2 in the unirradiated patient. Neurosurgery. 2018;83(1):38\u201342."},{"issue":"2","key":"b3","series-title":"Pediatr Blood Cancer","first-page":"e27520","article-title":"Malignant peripheral nerve sheath tumor: transformation in a patient with neurofibromatosis type 2","volume":"66","author":"Agresta L","year":"2019","unstructured":"Agresta L, Salloum R, Hummel TR,  Malignant peripheral nerve sheath tumor: transformation in a patient with neurofibromatosis type 2. Pediatr Blood Cancer. 2019;66(2):e27520."},{"issue":"5","key":"b4","series-title":"J Neurosurg","first-page":"1120","article-title":"Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation","volume":"125","author":"Carlson ML","year":"2016","unstructured":"Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ. Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation. J Neurosurg. 2016;125(5):1120\u20131129."},{"issue":"1","key":"b5","series-title":"Neurooncol Adv","first-page":"vdad025","article-title":"Radiation treatment of benign tumors in NF2-related-schwannomatosis: a national study of 266 irradiated patients showing a significant increase in malignancy\/malignant progression","volume":"5","author":"Evans DG","year":"2023","unstructured":"Evans DG, Halliday D, Obholzer R,  Radiation treatment of benign tumors in NF2-related-schwannomatosis: a national study of 266 irradiated patients showing a significant increase in malignancy\/malignant progression. Neurooncol Adv. 2023;5(1):vdad025."},{"issue":"5","key":"b6","series-title":"Pediatr Pathol Mol Med","first-page":"375","article-title":"Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis 2: a case report and review of the literature","volume":"22","author":"Chen Y","year":"2003","unstructured":"Chen Y, Diamond AS, Vaheesan KR, Schneider S, Valderrama E. Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis 2: a case report and review of the literature. Pediatr Pathol Mol Med. 2003;22(5):375\u2013381."},{"issue":"1","key":"b7","series-title":"World J Surg Oncol","first-page":"257","article-title":"Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis","volume":"18","author":"Cai Z","year":"2020","unstructured":"Cai Z, Tang X, Liang H, Yang R, Yan T, Guo W. Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis. World J Surg Oncol. 2020;18(1):257."},{"key":"b8","series-title":"Surveillance, Epidemiology, and End Results (SEER) Program","article-title":"SEER*Stat Database: Incidence - SEER Research Limited-Field Data, 22 Registries (excl IL and MA), Nov 2023 Sub (2000-2021)- Linked To County Attributes - Time Dependent (1990-2022) Income\/Rurality, 1969-2022 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, released April 2024, based on the November 2023 submission","unstructured":"SEER*Stat Database: Incidence - SEER Research Limited-Field Data, 22 Registries (excl IL and MA), Nov 2023 Sub (2000-2021)- Linked To County Attributes - Time Dependent (1990-2022) Income\/Rurality, 1969-2022 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, released April 2024, based on the November 2023 submission. Surveillance, Epidemiology, and End Results (SEER) Program. Accessed July 24, 2025. http:\/\/www.seer.cancer.gov"},{"issue":"4","key":"b9","series-title":"Histopathology","first-page":"379","article-title":"Epithelioid sarcoma arising in a patient with neurofibromatosis type 2","volume":"25","author":"Rose DS","year":"1994","unstructured":"Rose DS, Fisher C, Smith ME. Epithelioid sarcoma arising in a patient with neurofibromatosis type 2. Histopathology. 1994;25(4):379\u2013380."},{"issue":"2","key":"b10","series-title":"Clin Neuropathol","first-page":"115","article-title":"Malignant peripheral nerve sheath tumors developing multifocally in the central nervous system in a patient with neurofibromatosis type 2","volume":"17","author":"Higami Y","year":"1998","unstructured":"Higami Y, Shimokawa I, Kishikawa M,  Malignant peripheral nerve sheath tumors developing multifocally in the central nervous system in a patient with neurofibromatosis type 2. Clin Neuropathol. 1998;17(2):115\u2013120."},{"issue":"Suppl 1","key":"b11","series-title":"Stereotact Funct Neurosurg","first-page":"65","article-title":"Long-term complications following gamma knife radiosurgery of vestibular schwannomas","volume":"70","author":"Nor\u00e9n G","year":"1998","unstructured":"Nor\u00e9n G. Long-term complications following gamma knife radiosurgery of vestibular schwannomas. Stereotact Funct Neurosurg. 1998;70Suppl 1:65\u201373."},{"issue":"3","key":"b12","series-title":"Br J Neurosurg","first-page":"284","article-title":"Malignancy in a vestibular schwannoma. Report of a case with central neurofibromatosis, treated by both stereotactic radiosurgery and surgical excision, with a review of the literature","volume":"16","author":"Bari ME","year":"2002","unstructured":"Bari ME, Forster DMC, Kemeny AA, Walton L, Hardy D, Anderson JR. Malignancy in a vestibular schwannoma. Report of a case with central neurofibromatosis, treated by both stereotactic radiosurgery and surgical excision, with a review of the literature. Br J Neurosurg. 2002;16(3):284\u2013289."},{"issue":"1","key":"b13","series-title":"Clin Neuropathol","first-page":"23","article-title":"Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves","volume":"22","author":"Suresh TN","year":"2003","unstructured":"Suresh TN, Mahadevan A, Chandrashekhar Sagar B, Santosh V, Yasha TC, Shankar SK. Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves. Clin Neuropathol. 2003;22(1):23\u201329."},{"issue":"4","key":"b14","series-title":"Neurosurgery","first-page":"825","article-title":"Diagnosis and management of intracranial malignant peripheral nerve sheath tumors","volume":"62","author":"Chen L","year":"2008","unstructured":"Chen L, Mao Y, Chen H, Zhou LF. Diagnosis and management of intracranial malignant peripheral nerve sheath tumors. Neurosurgery. 2008;62(4):825\u2013832."},{"issue":"3","key":"b15","series-title":"Am J Surg Pathol","first-page":"325","article-title":"Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases","volume":"33","author":"Scheithauer BW","year":"2009","unstructured":"Scheithauer BW, Erdogan S, Rodriguez FJ,  Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol. 2009;33(3):325\u2013338."},{"issue":"2","key":"b16","series-title":"Int J Radiat Oncol Biol Phys","first-page":"513","article-title":"Gamma knife radiosurgery as a therapeutic strategy for intracranial sarcomatous metastases","volume":"76","author":"Flannery T","year":"2010","unstructured":"Flannery T, Kano H, Niranjan A,  Gamma knife radiosurgery as a therapeutic strategy for intracranial sarcomatous metastases. Int J Radiat Oncol Biol Phys. 2010;76(2):513\u2013519."},{"issue":"5","key":"b17","series-title":"Laryngoscope","first-page":"923","article-title":"Malignancy in vestibular schwannoma after stereotactic radiotherapy: a case report and review of the literature","volume":"121","author":"Tanbouzi Husseini S","year":"2011","unstructured":"Tanbouzi Husseini S, Piccirillo E, Taibah A, Paties CT, Rizzoli R, Sanna M. Malignancy in vestibular schwannoma after stereotactic radiotherapy: a case report and review of the literature. Laryngoscope. 2011;121(5):923\u2013928."},{"issue":"3","key":"b18","series-title":"J Neurosurg","first-page":"756","article-title":"Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up","volume":"114","author":"Gonzalvo A","year":"2011","unstructured":"Gonzalvo A, Fowler A, Cook RJ,  Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up. J Neurosurg. 2011;114(3):756\u2013762."},{"issue":"1","key":"b19","series-title":"Clin Sarcoma Res","first-page":"17","article-title":"Malignant peripheral nerve sheath tumours in inherited disease","volume":"2","author":"Evans DGR","year":"2012","unstructured":"Evans DGR, Huson SM, Birch JM. Malignant peripheral nerve sheath tumours in inherited disease. Clin Sarcoma Res. 2012;2(1):17."},{"issue":"1","key":"b20","series-title":"Am J Surg Pathol","first-page":"154","article-title":"Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with \"neuroblastoma-like\" schwannomatosis and a novel germline SMARCB1 mutation","volume":"36","author":"Carter JM","year":"2012","unstructured":"Carter JM, O\u2019Hara C, Dundas G,  Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with \"neuroblastoma-like\" schwannomatosis and a novel germline SMARCB1 mutation. Am J Surg Pathol. 2012;36(1):154\u2013160."},{"issue":"5","key":"b21","series-title":"Neuropathology","first-page":"541","article-title":"Malignant peripheral nerve sheath tumor of the trigeminal nerve: clinicopathologic features in a young adult patient","volume":"33","author":"Nakayama Y","year":"2013","unstructured":"Nakayama Y, Watanabe M, Suzuki K,  Malignant peripheral nerve sheath tumor of the trigeminal nerve: clinicopathologic features in a young adult patient. Neuropathology. 2013;33(5):541\u2013546."},{"issue":"1","key":"b22","series-title":"Turk Neurosurg","first-page":"135","article-title":"Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review","volume":"24","author":"Wu L","year":"2014","unstructured":"Wu L, Deng X, Yang C, Xu Y. Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review. Turk Neurosurg. 2014;24(1):135\u2013139."},{"issue":"1","key":"b23","series-title":"Br J Neurosurg","first-page":"37","article-title":"Causes of mortality in neurofibromatosis type 2","volume":"29","author":"Aboukais R","year":"2015","unstructured":"Aboukais R, Zairi F, Bonne NX,  Causes of mortality in neurofibromatosis type 2. Br J Neurosurg. 2015;29(1):37\u201340."},{"issue":"5","key":"b24","series-title":"Am J Surg Pathol","first-page":"673","article-title":"Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases","volume":"39","author":"Jo VY","year":"2015","unstructured":"Jo VY, Fletcher CDM. Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. Am J Surg Pathol. 2015;39(5):673\u2013682."},{"issue":"6","key":"b25","series-title":"Am J Surg Pathol","first-page":"835","article-title":"Recurrent SMARCB1 inactivation in epithelioid malignant peripheral nerve sheath tumors","volume":"43","author":"Schaefer IM","year":"2019","unstructured":"Schaefer IM, Dong F, Garcia EP, Fletcher CDM, Jo VY. Recurrent SMARCB1 inactivation in epithelioid malignant peripheral nerve sheath tumors. Am J Surg Pathol. 2019;43(6):835\u2013843."},{"issue":"suppl_1","key":"b26","series-title":"Am J Clin Pathol","first-page":"63","article-title":"A rare case of primary intracranial epithelioid sarcoma in a patient with neurofibromatosis type 2","volume":"146","author":"Baardsen E","year":"2016","unstructured":"Baardsen E, Thomas S, Suzuki G. A rare case of primary intracranial epithelioid sarcoma in a patient with neurofibromatosis type 2. Am J Clin Pathol. 2016;146(suppl_1):63."},{"key":"b27","series-title":"World Neurosurg","first-page":"335","article-title":"Malignant peripheral nerve sheath tumor arising in schwannomatosis with multiple lung metastases","volume":"119","author":"Ding Y","year":"2018","unstructured":"Ding Y, Rong H, Liu T,  Malignant peripheral nerve sheath tumor arising in schwannomatosis with multiple lung metastases. World Neurosurg. 2018;119:335\u2013339."},{"issue":"4","key":"b28","series-title":"Fam Cancer","first-page":"445","article-title":"Multiple primary malignancies associated with a germline SMARCB1 pathogenic variant","volume":"18","author":"Eelloo JA","year":"2019","unstructured":"Eelloo JA, Smith MJ, Bowers NL,  Multiple primary malignancies associated with a germline SMARCB1 pathogenic variant. Fam Cancer. 2019;18(4):445\u2013449."},{"issue":"1","key":"b29","series-title":"Fam Cancer","first-page":"97","article-title":"Sarcoma in neurofibromatosis 2: case report and review of the literature","volume":"18","author":"Linder C","year":"2019","unstructured":"Linder C, Smith MJ, Bulman M,  Sarcoma in neurofibromatosis 2: case report and review of the literature. Fam Cancer. 2019;18(1):97\u2013100."},{"issue":"4","key":"b30","series-title":"Acta Neuropathol","first-page":"505","article-title":"SMARCA4-associated schwannomatosis","volume":"145","author":"Chan-Pak-Choon F","year":"2023","unstructured":"Chan-Pak-Choon F, Roca C, Chong AS,  SMARCA4-associated schwannomatosis. Acta Neuropathol. 2023;145(4):505\u2013507."},{"issue":"1","key":"b31","series-title":"Acta Neuropathol","first-page":"22","article-title":"Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation","volume":"147","author":"Neyazi S","year":"2024","unstructured":"Neyazi S, Yamazawa E, Hack K,  Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation. Acta Neuropathol. 2024;147(1):22."},{"key":"b32","series-title":"J Neurosurg","article-title":"Malignant peripheral nerve sheath tumors in schwannomatosis: a case series","author":"Porche K","unstructured":"Porche K, Hayford KM, Gui C, Suppiah S, Spinner RJ. Malignant peripheral nerve sheath tumors in schwannomatosis: a case series. J Neurosurg. Published online November 14, 2025. doi:10.3171\/2025.6.JNS25872"},{"issue":"2","key":"b33","series-title":"Oncologist","first-page":"193","article-title":"Malignant peripheral nerve sheath tumors","volume":"19","author":"Farid M","year":"2014","unstructured":"Farid M, Demicco EG, Garcia R,  Malignant peripheral nerve sheath tumors. Oncologist. 2014;19(2):193\u2013201."},{"issue":"8","key":"b34","series-title":"J Natl Cancer Inst","first-page":"djx124","article-title":"Neurofibromatosis type 1\u2013associated MPNST state of the science: outlining a research agenda for the future","volume":"109","author":"Reilly KM","year":"2017","unstructured":"Reilly KM, Kim A, Blakely J,  Neurofibromatosis type 1\u2013associated MPNST state of the science: outlining a research agenda for the future. J Natl Cancer Inst. 2017;109(8):djx124."},{"key":"b35","series-title":"Annu Rev Pathol","first-page":"145","article-title":"SWI\/SNF chromatin remodeling and human malignancies","volume":"10","author":"Masliah-Planchon J","year":"2015","unstructured":"Masliah-Planchon J, Bi\u00e8che I, Guinebreti\u00e8re JM, Bourdeaut F, Delattre O. SWI\/SNF chromatin remodeling and human malignancies. Annu Rev Pathol. 2015;10:145\u2013171."},{"issue":"6689","key":"b36","series-title":"Nature","first-page":"203","article-title":"Truncating mutations of hSNF5\/INI1 in aggressive paediatric cancer","volume":"394","author":"Versteege I","year":"1998","unstructured":"Versteege I, S\u00e9venet N, Lange J,  Truncating mutations of hSNF5\/INI1 in aggressive paediatric cancer. Nature. 1998;394(6689):203\u2013206."},{"issue":"11","key":"b37","series-title":"J Neurol Neurosurg Psychiatry","first-page":"1215","article-title":"Schwannomatosis: a genetic and epidemiological study","volume":"89","author":"Evans DG","year":"2018","unstructured":"Evans DG, Bowers NL, Tobi S,  Schwannomatosis: a genetic and epidemiological study. J Neurol Neurosurg Psychiatry. 2018;89(11):1215\u20131219."},{"issue":"304","key":"b38","series-title":"Q J Med","first-page":"603","article-title":"A clinical study of type 2 neurofibromatosis","volume":"84","author":"Evans DG","year":"1992","unstructured":"Evans DG, Huson SM, Donnai D,  A clinical study of type 2 neurofibromatosis. Q J Med. 1992;84(304):603\u2013618."},{"issue":"2","key":"b39","series-title":"Am J Med Genet A","first-page":"327","article-title":"Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service","volume":"152A","author":"Evans DG","year":"2010","unstructured":"Evans DG, Howard E, Giblin C,  Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A. 2010;152A(2):327\u2013332."},{"issue":"7","key":"b40","series-title":"Cancers (Basel)","first-page":"1930","article-title":"Diagnosis and treatment of peripheral and cranial nerve tumors with expert recommendations: an EUropean Network for RAre CANcers (EURACAN) Initiative","volume":"15","author":"Pellerino A","year":"2023","unstructured":"Pellerino A, Verdijk RM, Nichelli L, Andratschke NH, Idbaih A, Goldbrunner R. Diagnosis and treatment of peripheral and cranial nerve tumors with expert recommendations: an EUropean Network for RAre CANcers (EURACAN) Initiative. Cancers (Basel). 2023;15(7):1930."},{"issue":"1","key":"b41","series-title":"Neurooncol Adv","first-page":"vdac005","article-title":"Clinical trials targeting neurofibromatoses-associated tumors: a systematic review","volume":"4","author":"Roman Souza G","year":"2022","unstructured":"Roman Souza G, Abdalla A, Mahadevan D. Clinical trials targeting neurofibromatoses-associated tumors: a systematic review. Neurooncol Adv. 2022;4(1):vdac005."},{"issue":"2","key":"b42","series-title":"Neurosurgery","first-page":"e15","article-title":"Commentary: surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2","volume":"92","author":"Bhandarkar AR","year":"2023","unstructured":"Bhandarkar AR, Spinner RJ. Commentary: surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2. Neurosurgery. 2023;92(2):e15\u2013e16."},{"issue":"3","key":"b43","series-title":"Am J Cancer Res","first-page":"856","article-title":"Polo-like kinase 1 as a therapeutic target for malignant peripheral nerve sheath tumors (MPNST) and schwannomas","volume":"10","author":"Guo J","year":"2020","unstructured":"Guo J, Chaney KE, Choi K,  Polo-like kinase 1 as a therapeutic target for malignant peripheral nerve sheath tumors (MPNST) and schwannomas. Am J Cancer Res. 2020;10(3):856\u2013869."},{"issue":"2","key":"b44","series-title":"Neurosurgery","first-page":"317","article-title":"Surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2","volume":"92","author":"Peyre M","year":"2023","unstructured":"Peyre M, Tran S, Parfait B, Bernat I, Bielle F, Kalamarides M. Surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2. Neurosurgery. 2023;92(2):317\u2013328."},{"issue":"5","key":"b45","series-title":"Cancer Res","first-page":"1573","article-title":"International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis","volume":"62","author":"Ferner RE","year":"2002","unstructured":"Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res. 2002;62(5):1573\u20131577."},{"issue":"1","key":"b46","series-title":"Clin Trials","first-page":"51","article-title":"Gene-targeted therapy for neurofibromatosis and schwannomatosis: the path to clinical trials","volume":"21","author":"Staedtke V","year":"2024","unstructured":"Staedtke V, Anstett K, Bedwell D,  Gene-targeted therapy for neurofibromatosis and schwannomatosis: the path to clinical trials. Clin Trials. 2024;21(1):51\u201366."},{"issue":"5","key":"b47","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 JA","year":"1993","unstructured":"Trofatter JA, MacCollin MM, Rutter JL,  A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell. 1993;72(5):791\u2013800."},{"issue":"1","key":"b48","series-title":"Neurooncol Adv","first-page":"vdac072","article-title":"Validation of Bromodomain and Extraterminal proteins as therapeutic targets in neurofibromatosis type 2","volume":"4","author":"Doherty J","year":"2022","unstructured":"Doherty J, Mandati V, Gonz\u00e1lez Rodriguez MA,  Validation of Bromodomain and Extraterminal proteins as therapeutic targets in neurofibromatosis type 2. Neurooncol Adv. 2022;4(1):vdac072."},{"issue":"5","key":"b49","series-title":"Pediatr Neurosurg","first-page":"267","article-title":"Updates in the management of central and peripheral nervous system tumors among patients with neurofibromatosis type 1 and neurofibromatosis type 2","volume":"58","author":"Kotch C","year":"2023","unstructured":"Kotch C, Brosius SN, De Raedt T, Fisher MJ. Updates in the management of central and peripheral nervous system tumors among patients with neurofibromatosis type 1 and neurofibromatosis type 2. Pediatr Neurosurg. 2023;58(5):267\u2013280."},{"issue":"7","key":"b50","series-title":"Eur J Hum Genet","first-page":"812","article-title":"ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis","volume":"30","author":"Evans DG","year":"2022","unstructured":"Evans DG, Mostaccioli S, Pang D,  ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis. Eur J Hum Genet. 2022;30(7):812\u2013817."},{"issue":"3-4","key":"b51","series-title":"Crit Rev Oncog","first-page":"199","article-title":"Biology and treatment of rhabdoid tumor","volume":"20","author":"Geller JI","year":"2015","unstructured":"Geller JI, Roth JJ, Biegel JA. Biology and treatment of rhabdoid tumor. Crit Rev Oncog. 2015;20(3-4):199\u2013216."},{"issue":"16_suppl","key":"b52","series-title":"J Clin Oncol","first-page":"TPS10066","article-title":"Phase 1\/2 study of tiragolumab and atezolizumab in patients with relapsed or refractory SMARCB1 or SMARCA4 deficient tumors","volume":"41","author":"Wedekind MF","year":"2023","unstructured":"Wedekind MF, Akshintala S, Widemann BC,  Phase 1\/2 study of tiragolumab and atezolizumab in patients with relapsed or refractory SMARCB1 or SMARCA4 deficient tumors. J Clin Oncol. 2023;41(16_suppl):TPS10066."},{"key":"b1","series-title":"Central Nervous System Tumours. WHO Classification of Tumours, 5th Edition, Volume 6","year":"2021","unstructured":"Central Nervous System Tumours. WHO Classification of Tumours, 5th Edition, Volume 6. World Health Organization; 2021."},{"issue":"1","key":"b2","series-title":"Neurosurgery","first-page":"38","article-title":"Malignant peripheral nerve sheath tumors are not a feature of neurofibromatosis type 2 in the unirradiated patient","volume":"83","author":"King AT","year":"2018","unstructured":"King AT, Rutherford SA, Hammerbeck-Ward C,  Malignant peripheral nerve sheath tumors are not a feature of neurofibromatosis type 2 in the unirradiated patient. Neurosurgery. 2018;83(1):38\u201342."},{"issue":"2","key":"b3","series-title":"Pediatr Blood Cancer","first-page":"e27520","article-title":"Malignant peripheral nerve sheath tumor: transformation in a patient with neurofibromatosis type 2","volume":"66","author":"Agresta L","year":"2019","unstructured":"Agresta L, Salloum R, Hummel TR,  Malignant peripheral nerve sheath tumor: transformation in a patient with neurofibromatosis type 2. Pediatr Blood Cancer. 2019;66(2):e27520."},{"issue":"5","key":"b4","series-title":"J Neurosurg","first-page":"1120","article-title":"Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation","volume":"125","author":"Carlson ML","year":"2016","unstructured":"Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ. Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation. J Neurosurg. 2016;125(5):1120\u20131129."},{"issue":"1","key":"b5","series-title":"Neurooncol Adv","first-page":"vdad025","article-title":"Radiation treatment of benign tumors in NF2-related-schwannomatosis: a national study of 266 irradiated patients showing a significant increase in malignancy\/malignant progression","volume":"5","author":"Evans DG","year":"2023","unstructured":"Evans DG, Halliday D, Obholzer R,  Radiation treatment of benign tumors in NF2-related-schwannomatosis: a national study of 266 irradiated patients showing a significant increase in malignancy\/malignant progression. Neurooncol Adv. 2023;5(1):vdad025."},{"issue":"5","key":"b6","series-title":"Pediatr Pathol Mol Med","first-page":"375","article-title":"Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis 2: a case report and review of the literature","volume":"22","author":"Chen Y","year":"2003","unstructured":"Chen Y, Diamond AS, Vaheesan KR, Schneider S, Valderrama E. Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis 2: a case report and review of the literature. Pediatr Pathol Mol Med. 2003;22(5):375\u2013381."},{"issue":"1","key":"b7","series-title":"World J Surg Oncol","first-page":"257","article-title":"Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis","volume":"18","author":"Cai Z","year":"2020","unstructured":"Cai Z, Tang X, Liang H, Yang R, Yan T, Guo W. Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis. World J Surg Oncol. 2020;18(1):257."},{"key":"b8","series-title":"Surveillance, Epidemiology, and End Results (SEER) Program","article-title":"SEER*Stat Database: Incidence - SEER Research Limited-Field Data, 22 Registries (excl IL and MA), Nov 2023 Sub (2000-2021)- Linked To County Attributes - Time Dependent (1990-2022) Income\/Rurality, 1969-2022 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, released April 2024, based on the November 2023 submission","unstructured":"SEER*Stat Database: Incidence - SEER Research Limited-Field Data, 22 Registries (excl IL and MA), Nov 2023 Sub (2000-2021)- Linked To County Attributes - Time Dependent (1990-2022) Income\/Rurality, 1969-2022 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, released April 2024, based on the November 2023 submission. Surveillance, Epidemiology, and End Results (SEER) Program. Accessed July 24, 2025. http:\/\/www.seer.cancer.gov"},{"issue":"4","key":"b9","series-title":"Histopathology","first-page":"379","article-title":"Epithelioid sarcoma arising in a patient with neurofibromatosis type 2","volume":"25","author":"Rose DS","year":"1994","unstructured":"Rose DS, Fisher C, Smith ME. Epithelioid sarcoma arising in a patient with neurofibromatosis type 2. Histopathology. 1994;25(4):379\u2013380."},{"issue":"2","key":"b10","series-title":"Clin Neuropathol","first-page":"115","article-title":"Malignant peripheral nerve sheath tumors developing multifocally in the central nervous system in a patient with neurofibromatosis type 2","volume":"17","author":"Higami Y","year":"1998","unstructured":"Higami Y, Shimokawa I, Kishikawa M,  Malignant peripheral nerve sheath tumors developing multifocally in the central nervous system in a patient with neurofibromatosis type 2. Clin Neuropathol. 1998;17(2):115\u2013120."},{"issue":"Suppl 1","key":"b11","series-title":"Stereotact Funct Neurosurg","first-page":"65","article-title":"Long-term complications following gamma knife radiosurgery of vestibular schwannomas","volume":"70","author":"Nor\u00e9n G","year":"1998","unstructured":"Nor\u00e9n G. Long-term complications following gamma knife radiosurgery of vestibular schwannomas. Stereotact Funct Neurosurg. 1998;70Suppl 1:65\u201373."},{"issue":"3","key":"b12","series-title":"Br J Neurosurg","first-page":"284","article-title":"Malignancy in a vestibular schwannoma. Report of a case with central neurofibromatosis, treated by both stereotactic radiosurgery and surgical excision, with a review of the literature","volume":"16","author":"Bari ME","year":"2002","unstructured":"Bari ME, Forster DMC, Kemeny AA, Walton L, Hardy D, Anderson JR. Malignancy in a vestibular schwannoma. Report of a case with central neurofibromatosis, treated by both stereotactic radiosurgery and surgical excision, with a review of the literature. Br J Neurosurg. 2002;16(3):284\u2013289."},{"issue":"1","key":"b13","series-title":"Clin Neuropathol","first-page":"23","article-title":"Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves","volume":"22","author":"Suresh TN","year":"2003","unstructured":"Suresh TN, Mahadevan A, Chandrashekhar Sagar B, Santosh V, Yasha TC, Shankar SK. Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves. Clin Neuropathol. 2003;22(1):23\u201329."},{"issue":"4","key":"b14","series-title":"Neurosurgery","first-page":"825","article-title":"Diagnosis and management of intracranial malignant peripheral nerve sheath tumors","volume":"62","author":"Chen L","year":"2008","unstructured":"Chen L, Mao Y, Chen H, Zhou LF. Diagnosis and management of intracranial malignant peripheral nerve sheath tumors. Neurosurgery. 2008;62(4):825\u2013832."},{"issue":"3","key":"b15","series-title":"Am J Surg Pathol","first-page":"325","article-title":"Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases","volume":"33","author":"Scheithauer BW","year":"2009","unstructured":"Scheithauer BW, Erdogan S, Rodriguez FJ,  Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol. 2009;33(3):325\u2013338."},{"issue":"2","key":"b16","series-title":"Int J Radiat Oncol Biol Phys","first-page":"513","article-title":"Gamma knife radiosurgery as a therapeutic strategy for intracranial sarcomatous metastases","volume":"76","author":"Flannery T","year":"2010","unstructured":"Flannery T, Kano H, Niranjan A,  Gamma knife radiosurgery as a therapeutic strategy for intracranial sarcomatous metastases. Int J Radiat Oncol Biol Phys. 2010;76(2):513\u2013519."},{"issue":"5","key":"b17","series-title":"Laryngoscope","first-page":"923","article-title":"Malignancy in vestibular schwannoma after stereotactic radiotherapy: a case report and review of the literature","volume":"121","author":"Tanbouzi Husseini S","year":"2011","unstructured":"Tanbouzi Husseini S, Piccirillo E, Taibah A, Paties CT, Rizzoli R, Sanna M. Malignancy in vestibular schwannoma after stereotactic radiotherapy: a case report and review of the literature. Laryngoscope. 2011;121(5):923\u2013928."},{"issue":"3","key":"b18","series-title":"J Neurosurg","first-page":"756","article-title":"Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up","volume":"114","author":"Gonzalvo A","year":"2011","unstructured":"Gonzalvo A, Fowler A, Cook RJ,  Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up. J Neurosurg. 2011;114(3):756\u2013762."},{"issue":"1","key":"b19","series-title":"Clin Sarcoma Res","first-page":"17","article-title":"Malignant peripheral nerve sheath tumours in inherited disease","volume":"2","author":"Evans DGR","year":"2012","unstructured":"Evans DGR, Huson SM, Birch JM. Malignant peripheral nerve sheath tumours in inherited disease. Clin Sarcoma Res. 2012;2(1):17."},{"issue":"1","key":"b20","series-title":"Am J Surg Pathol","first-page":"154","article-title":"Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with \"neuroblastoma-like\" schwannomatosis and a novel germline SMARCB1 mutation","volume":"36","author":"Carter JM","year":"2012","unstructured":"Carter JM, O\u2019Hara C, Dundas G,  Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with \"neuroblastoma-like\" schwannomatosis and a novel germline SMARCB1 mutation. Am J Surg Pathol. 2012;36(1):154\u2013160."},{"issue":"5","key":"b21","series-title":"Neuropathology","first-page":"541","article-title":"Malignant peripheral nerve sheath tumor of the trigeminal nerve: clinicopathologic features in a young adult patient","volume":"33","author":"Nakayama Y","year":"2013","unstructured":"Nakayama Y, Watanabe M, Suzuki K,  Malignant peripheral nerve sheath tumor of the trigeminal nerve: clinicopathologic features in a young adult patient. Neuropathology. 2013;33(5):541\u2013546."},{"issue":"1","key":"b22","series-title":"Turk Neurosurg","first-page":"135","article-title":"Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review","volume":"24","author":"Wu L","year":"2014","unstructured":"Wu L, Deng X, Yang C, Xu Y. Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review. Turk Neurosurg. 2014;24(1):135\u2013139."},{"issue":"1","key":"b23","series-title":"Br J Neurosurg","first-page":"37","article-title":"Causes of mortality in neurofibromatosis type 2","volume":"29","author":"Aboukais R","year":"2015","unstructured":"Aboukais R, Zairi F, Bonne NX,  Causes of mortality in neurofibromatosis type 2. Br J Neurosurg. 2015;29(1):37\u201340."},{"issue":"5","key":"b24","series-title":"Am J Surg Pathol","first-page":"673","article-title":"Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases","volume":"39","author":"Jo VY","year":"2015","unstructured":"Jo VY, Fletcher CDM. Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. Am J Surg Pathol. 2015;39(5):673\u2013682."},{"issue":"6","key":"b25","series-title":"Am J Surg Pathol","first-page":"835","article-title":"Recurrent SMARCB1 inactivation in epithelioid malignant peripheral nerve sheath tumors","volume":"43","author":"Schaefer IM","year":"2019","unstructured":"Schaefer IM, Dong F, Garcia EP, Fletcher CDM, Jo VY. Recurrent SMARCB1 inactivation in epithelioid malignant peripheral nerve sheath tumors. Am J Surg Pathol. 2019;43(6):835\u2013843."},{"issue":"suppl_1","key":"b26","series-title":"Am J Clin Pathol","first-page":"63","article-title":"A rare case of primary intracranial epithelioid sarcoma in a patient with neurofibromatosis type 2","volume":"146","author":"Baardsen E","year":"2016","unstructured":"Baardsen E, Thomas S, Suzuki G. A rare case of primary intracranial epithelioid sarcoma in a patient with neurofibromatosis type 2. Am J Clin Pathol. 2016;146(suppl_1):63."},{"key":"b27","series-title":"World Neurosurg","first-page":"335","article-title":"Malignant peripheral nerve sheath tumor arising in schwannomatosis with multiple lung metastases","volume":"119","author":"Ding Y","year":"2018","unstructured":"Ding Y, Rong H, Liu T,  Malignant peripheral nerve sheath tumor arising in schwannomatosis with multiple lung metastases. World Neurosurg. 2018;119:335\u2013339."},{"issue":"4","key":"b28","series-title":"Fam Cancer","first-page":"445","article-title":"Multiple primary malignancies associated with a germline SMARCB1 pathogenic variant","volume":"18","author":"Eelloo JA","year":"2019","unstructured":"Eelloo JA, Smith MJ, Bowers NL,  Multiple primary malignancies associated with a germline SMARCB1 pathogenic variant. Fam Cancer. 2019;18(4):445\u2013449."},{"issue":"1","key":"b29","series-title":"Fam Cancer","first-page":"97","article-title":"Sarcoma in neurofibromatosis 2: case report and review of the literature","volume":"18","author":"Linder C","year":"2019","unstructured":"Linder C, Smith MJ, Bulman M,  Sarcoma in neurofibromatosis 2: case report and review of the literature. Fam Cancer. 2019;18(1):97\u2013100."},{"issue":"4","key":"b30","series-title":"Acta Neuropathol","first-page":"505","article-title":"SMARCA4-associated schwannomatosis","volume":"145","author":"Chan-Pak-Choon F","year":"2023","unstructured":"Chan-Pak-Choon F, Roca C, Chong AS,  SMARCA4-associated schwannomatosis. Acta Neuropathol. 2023;145(4):505\u2013507."},{"issue":"1","key":"b31","series-title":"Acta Neuropathol","first-page":"22","article-title":"Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation","volume":"147","author":"Neyazi S","year":"2024","unstructured":"Neyazi S, Yamazawa E, Hack K,  Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation. Acta Neuropathol. 2024;147(1):22."},{"key":"b32","series-title":"J Neurosurg","article-title":"Malignant peripheral nerve sheath tumors in schwannomatosis: a case series","author":"Porche K","unstructured":"Porche K, Hayford KM, Gui C, Suppiah S, Spinner RJ. Malignant peripheral nerve sheath tumors in schwannomatosis: a case series. J Neurosurg. Published online November 14, 2025. doi:10.3171\/2025.6.JNS25872"},{"issue":"2","key":"b33","series-title":"Oncologist","first-page":"193","article-title":"Malignant peripheral nerve sheath tumors","volume":"19","author":"Farid M","year":"2014","unstructured":"Farid M, Demicco EG, Garcia R,  Malignant peripheral nerve sheath tumors. Oncologist. 2014;19(2):193\u2013201."},{"issue":"8","key":"b34","series-title":"J Natl Cancer Inst","first-page":"djx124","article-title":"Neurofibromatosis type 1\u2013associated MPNST state of the science: outlining a research agenda for the future","volume":"109","author":"Reilly KM","year":"2017","unstructured":"Reilly KM, Kim A, Blakely J,  Neurofibromatosis type 1\u2013associated MPNST state of the science: outlining a research agenda for the future. J Natl Cancer Inst. 2017;109(8):djx124."},{"key":"b35","series-title":"Annu Rev Pathol","first-page":"145","article-title":"SWI\/SNF chromatin remodeling and human malignancies","volume":"10","author":"Masliah-Planchon J","year":"2015","unstructured":"Masliah-Planchon J, Bi\u00e8che I, Guinebreti\u00e8re JM, Bourdeaut F, Delattre O. SWI\/SNF chromatin remodeling and human malignancies. Annu Rev Pathol. 2015;10:145\u2013171."},{"issue":"6689","key":"b36","series-title":"Nature","first-page":"203","article-title":"Truncating mutations of hSNF5\/INI1 in aggressive paediatric cancer","volume":"394","author":"Versteege I","year":"1998","unstructured":"Versteege I, S\u00e9venet N, Lange J,  Truncating mutations of hSNF5\/INI1 in aggressive paediatric cancer. Nature. 1998;394(6689):203\u2013206."},{"issue":"11","key":"b37","series-title":"J Neurol Neurosurg Psychiatry","first-page":"1215","article-title":"Schwannomatosis: a genetic and epidemiological study","volume":"89","author":"Evans DG","year":"2018","unstructured":"Evans DG, Bowers NL, Tobi S,  Schwannomatosis: a genetic and epidemiological study. J Neurol Neurosurg Psychiatry. 2018;89(11):1215\u20131219."},{"issue":"304","key":"b38","series-title":"Q J Med","first-page":"603","article-title":"A clinical study of type 2 neurofibromatosis","volume":"84","author":"Evans DG","year":"1992","unstructured":"Evans DG, Huson SM, Donnai D,  A clinical study of type 2 neurofibromatosis. Q J Med. 1992;84(304):603\u2013618."},{"issue":"2","key":"b39","series-title":"Am J Med Genet A","first-page":"327","article-title":"Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service","volume":"152A","author":"Evans DG","year":"2010","unstructured":"Evans DG, Howard E, Giblin C,  Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A. 2010;152A(2):327\u2013332."},{"issue":"7","key":"b40","series-title":"Cancers (Basel)","first-page":"1930","article-title":"Diagnosis and treatment of peripheral and cranial nerve tumors with expert recommendations: an EUropean Network for RAre CANcers (EURACAN) Initiative","volume":"15","author":"Pellerino A","year":"2023","unstructured":"Pellerino A, Verdijk RM, Nichelli L, Andratschke NH, Idbaih A, Goldbrunner R. Diagnosis and treatment of peripheral and cranial nerve tumors with expert recommendations: an EUropean Network for RAre CANcers (EURACAN) Initiative. Cancers (Basel). 2023;15(7):1930."},{"issue":"1","key":"b41","series-title":"Neurooncol Adv","first-page":"vdac005","article-title":"Clinical trials targeting neurofibromatoses-associated tumors: a systematic review","volume":"4","author":"Roman Souza G","year":"2022","unstructured":"Roman Souza G, Abdalla A, Mahadevan D. Clinical trials targeting neurofibromatoses-associated tumors: a systematic review. Neurooncol Adv. 2022;4(1):vdac005."},{"issue":"2","key":"b42","series-title":"Neurosurgery","first-page":"e15","article-title":"Commentary: surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2","volume":"92","author":"Bhandarkar AR","year":"2023","unstructured":"Bhandarkar AR, Spinner RJ. Commentary: surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2. Neurosurgery. 2023;92(2):e15\u2013e16."},{"issue":"3","key":"b43","series-title":"Am J Cancer Res","first-page":"856","article-title":"Polo-like kinase 1 as a therapeutic target for malignant peripheral nerve sheath tumors (MPNST) and schwannomas","volume":"10","author":"Guo J","year":"2020","unstructured":"Guo J, Chaney KE, Choi K,  Polo-like kinase 1 as a therapeutic target for malignant peripheral nerve sheath tumors (MPNST) and schwannomas. Am J Cancer Res. 2020;10(3):856\u2013869."},{"issue":"2","key":"b44","series-title":"Neurosurgery","first-page":"317","article-title":"Surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2","volume":"92","author":"Peyre M","year":"2023","unstructured":"Peyre M, Tran S, Parfait B, Bernat I, Bielle F, Kalamarides M. Surgical management of peripheral nerve pathology in patients with neurofibromatosis type 2. Neurosurgery. 2023;92(2):317\u2013328."},{"issue":"5","key":"b45","series-title":"Cancer Res","first-page":"1573","article-title":"International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis","volume":"62","author":"Ferner RE","year":"2002","unstructured":"Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res. 2002;62(5):1573\u20131577."},{"issue":"1","key":"b46","series-title":"Clin Trials","first-page":"51","article-title":"Gene-targeted therapy for neurofibromatosis and schwannomatosis: the path to clinical trials","volume":"21","author":"Staedtke V","year":"2024","unstructured":"Staedtke V, Anstett K, Bedwell D,  Gene-targeted therapy for neurofibromatosis and schwannomatosis: the path to clinical trials. Clin Trials. 2024;21(1):51\u201366."},{"issue":"5","key":"b47","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 JA","year":"1993","unstructured":"Trofatter JA, MacCollin MM, Rutter JL,  A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell. 1993;72(5):791\u2013800."},{"issue":"1","key":"b48","series-title":"Neurooncol Adv","first-page":"vdac072","article-title":"Validation of Bromodomain and Extraterminal proteins as therapeutic targets in neurofibromatosis type 2","volume":"4","author":"Doherty J","year":"2022","unstructured":"Doherty J, Mandati V, Gonz\u00e1lez Rodriguez MA,  Validation of Bromodomain and Extraterminal proteins as therapeutic targets in neurofibromatosis type 2. Neurooncol Adv. 2022;4(1):vdac072."},{"issue":"5","key":"b49","series-title":"Pediatr Neurosurg","first-page":"267","article-title":"Updates in the management of central and peripheral nervous system tumors among patients with neurofibromatosis type 1 and neurofibromatosis type 2","volume":"58","author":"Kotch C","year":"2023","unstructured":"Kotch C, Brosius SN, De Raedt T, Fisher MJ. Updates in the management of central and peripheral nervous system tumors among patients with neurofibromatosis type 1 and neurofibromatosis type 2. Pediatr Neurosurg. 2023;58(5):267\u2013280."},{"issue":"7","key":"b50","series-title":"Eur J Hum Genet","first-page":"812","article-title":"ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis","volume":"30","author":"Evans DG","year":"2022","unstructured":"Evans DG, Mostaccioli S, Pang D,  ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis. Eur J Hum Genet. 2022;30(7):812\u2013817."},{"issue":"3-4","key":"b51","series-title":"Crit Rev Oncog","first-page":"199","article-title":"Biology and treatment of rhabdoid tumor","volume":"20","author":"Geller JI","year":"2015","unstructured":"Geller JI, Roth JJ, Biegel JA. Biology and treatment of rhabdoid tumor. Crit Rev Oncog. 2015;20(3-4):199\u2013216."},{"issue":"16_suppl","key":"b52","series-title":"J Clin Oncol","first-page":"TPS10066","article-title":"Phase 1\/2 study of tiragolumab and atezolizumab in patients with relapsed or refractory SMARCB1 or SMARCA4 deficient tumors","volume":"41","author":"Wedekind MF","year":"2023","unstructured":"Wedekind MF, Akshintala S, Widemann BC,  Phase 1\/2 study of tiragolumab and atezolizumab in patients with relapsed or refractory SMARCB1 or SMARCA4 deficient tumors. J Clin Oncol. 2023;41(16_suppl):TPS10066."}],"container-title":["Journal of Neurosurgery"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/144\/3\/article-p642.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/144\/3\/article-p642.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/144\/3\/article-p642.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T14:31:45Z","timestamp":1772721105000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/144\/3\/article-p642.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,1]]},"references-count":104,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3171\/2025.6.jns251508","relation":{},"ISSN":["0022-3085","1933-0693"],"issn-type":[{"value":"0022-3085","type":"print"},{"value":"1933-0693","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,1]]}}}