{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T20:58:54Z","timestamp":1790629134815,"version":"4.1.0"},"reference-count":48,"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 predominantly associated with neurofibromatosis type 1 (NF1). However, their occurrence within NF type 2 (NF2)\u2013related and SMARCB1-related schwannomatosis remains rare and poorly characterized. Accurate and timely diagnosis is challenging due to clinical and radiological similarities with benign schwannomas, potentially delaying appropriate management and impacting outcomes. This study presents an institutional case series aiming to better characterize the clinical presentations specifically for MPNSTs arising in patients with NF2- and SMARCB1-related schwannomatosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>This retrospective case series included patients diagnosed with schwannomatosis who subsequently developed histopathologically confirmed MPNSTs. Conducted at Mayo Clinic (Rochester, Minnesota) and University Health Network (Toronto, Canada), the study spanned January 1, 2003, to November 1, 2024. Patients were selected based on clinical or genetic diagnoses of schwannomatosis using International Consensus Group on Neurofibromatosis Diagnostic Criteria. Comprehensive clinical, radiological, and pathological data, including demographics, presenting symptoms, tumor features (size, location, immunohistochemistry, genetics), treatments (resection, chemotherapy, radiotherapy), and follow-up outcomes were extracted from electronic medical records.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>The authors identified 8 patients with a mean age of 36 years, half of whom had NF2-related schwannomatosis. The most common presenting symptom was pain (71%). The mean tumor size was 8.2 \u00b1 7.0 cm, with gross-total resection being the most common surgical treatment (67%), typically supplemented with radiation therapy (78%). More than half of the patients (56%) exhibited metastatic disease, and all tumors with reported grading were F\u00e9d\u00e9ration Nationale des Centres de Lutte Contre le Cancer grade 3. No tumors were associated with LZTR1 mutations. One notable case involved a 39-year-old male with SMARCB1-related schwannomatosis who had a femoral MPNST resected 13 years prior and subsequently developed a sciatic nerve MPNST that was managed successfully with neoadjuvant chemotherapy, radiation therapy, and negative margin resection. Of note, the second lesion arose from a hybrid neurofibroma\/schwannoma. Another case, a 33-year-old male with SMARCB1-related schwannomatosis, had an incidental finding of epithelioid MPNST in a minimally symptomatic small palmar lesion.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>This case series highlights that MPNSTs, although uncommon, can arise in NF2- and SMARCB1-related schwannomatosis without prior radiation exposure, presenting significant diagnostic challenges due to their similarity to benign schwannomas. The findings underscore the importance of maintaining clinical vigilance and employing individualized management strategies, balancing thorough resection with the preservation of function and patient quality of life.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2025.6.jns25872","type":"journal-article","created":{"date-parts":[[2025,11,15]],"date-time":"2025-11-15T01:41:58Z","timestamp":1763170918000},"page":"633-641","source":"Crossref","is-referenced-by-count":2,"title":["Malignant peripheral nerve sheath tumors in schwannomatosis: a case series"],"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":"Department of Neurosurgery, University Health Network, University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suganth","family":"Suppiah","sequence":"additional","affiliation":[{"name":"Department 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. 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Pediatr Blood Cancer. 2019;66(2):e27520."},{"issue":"1","key":"b3","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":"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":"9","key":"b7","series-title":"Genet Med","first-page":"1967","article-title":"Updated diagnostic criteria and nomenclature for neurofibromatosis type 2 and schwannomatosis: an international consensus recommendation","volume":"24","author":"Plotkin SR","year":"2022","unstructured":"Plotkin SR, Messiaen L, Legius E,  Updated diagnostic criteria and nomenclature for neurofibromatosis type 2 and schwannomatosis: an international consensus recommendation. Genet Med. 2022;24(9):1967\u20131977."},{"issue":"2","key":"b8","series-title":"Neurosurg Clin N Am","first-page":"145","article-title":"The neurofibromatoses: hereditary predisposition to multiple peripheral nerve tumors","volume":"15","author":"Brooks DG","year":"2004","unstructured":"Brooks DG. 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