{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T01:17:22Z","timestamp":1790299042194,"version":"4.1.0"},"reference-count":36,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,5,1]]},"abstract":"<jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>Schwannomatosis is a rare genetic predisposition to develop multiple nonintradermal schwannomas affecting the spine and peripheral nerves, excluding vestibular schwannomas. Schwannomatosis-associated schwannomas can present with pain or motor deficits, prompting resection. In this study, the authors reported the clinical characteristics, surgical interventions, and postoperative outcomes of patients with schwannomatosis-related schwannomas treated with resection.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>This was a retrospective cohort study of adult patients with schwannomatosis who had undergone resection of a symptomatic peripheral or spinal schwannoma at the authors\u2019 institution from September 2003 to June 2023. Data were extracted from electronic health records and provider notes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>A cohort of 85 patients (57.6% women) had a mean follow-up of 68.4 \u00b1 58.2 months after surgery. The mean age at the time of surgery was 44.6 \u00b1 16.5 years. Pain was the most common presenting symptom (94.1%), followed by motor weakness (36.4%) and sensory deficits (32.9%). Just under two-thirds of the patients (64.7%) had undergone prior surgery for schwannoma, 20 (36.4%) of whom had a prior resection in the same exact location. Gross-total resection (GTR) was achieved in 88.2% of cases and en bloc resection in 69.4%. There were significant improvements in pain, motor weakness, and sensory deficits at the last postoperative follow-up visit. There was local tumor recurrence in 14 patients (16.5%), and the overall mortality rate was 4.7%. Univariate and multivariate analyses identified GTR as the strongest predictor of a decreased risk of local recurrence (HR 0.09, 95% CI 0.02\u22120.48, p = 0.005).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>Patients with schwannomatosis commonly presented with neurological symptoms of pain, sensory deficits, or motor weakness associated with their schwannomas. Resection significantly improved their preoperative symptoms, and GTR was associated with a decreased risk of local recurrence.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2025.2.focus24751","type":"journal-article","created":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T06:39:34Z","timestamp":1746081574000},"page":"E2","source":"Crossref","is-referenced-by-count":4,"title":["Surgical management of schwannomas in schwannomatosis: a comprehensive analysis of clinical outcomes and determinants of local recurrence"],"prefix":"10.3171","volume":"58","author":[{"given":"Abdel-Hameed","family":"Al-Mistarehi","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jawad M.","family":"Khalifeh","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Avi N.","family":"Albert","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"},{"name":"Meharry Medical College, School of Medicine, Nashville, Tennessee; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carly","family":"Weber-Levine","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas","family":"Orlando","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Blanks","sequence":"additional","affiliation":[{"name":"School of Medicine, West Virginia University, Morgantown, West Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M.","family":"Hersh","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos G.","family":"Romo","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaishri","family":"Blakeley","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allan J.","family":"Belzberg","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Lubelski","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"3","key":"b1","series-title":"Brain Pathol","first-page":"205","article-title":"Schwannomas and their pathogenesis","volume":"24","author":"Hilton DA","year":"2014","unstructured":"Hilton DA, Hanemann CO. Schwannomas and their pathogenesis. Brain Pathol. 2014;24(3):205\u2013220."},{"issue":"11","key":"b2","series-title":"Int J Mol Sci","first-page":"5850","article-title":"Current understanding of neurofibromatosis type 1, 2, and schwannomatosis","volume":"22","author":"Tamura R","year":"2021","unstructured":"Tamura R. Current understanding of neurofibromatosis type 1, 2, and schwannomatosis. Int J Mol Sci. 2021;22(11):5850."},{"issue":"2","key":"b3","series-title":"Br J Neurosurg","first-page":"171","article-title":"What to know about schwannomatosis: a literature review","volume":"36","author":"Schraepen C","year":"2022","unstructured":"Schraepen C, Donkersloot P, Duyvendak W,  What to know about schwannomatosis: a literature review. Br J Neurosurg. 2022;36(2):171\u2013174."},{"issue":"7","key":"b4","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":"10","key":"b5","series-title":"Oncologist","first-page":"1317","article-title":"Clinical features of schwannomatosis: a retrospective analysis of 87 patients","volume":"17","author":"Merker VL","year":"2012","unstructured":"Merker VL, Esparza S, Smith MJ, Stemmer-Rachamimov A, Plotkin SR. Clinical features of schwannomatosis: a retrospective analysis of 87 patients. Oncologist. 2012;17(10):1317\u20131322."},{"issue":"9","key":"b6","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":"6","key":"b7","series-title":"Med Clin North Am","first-page":"1035","article-title":"The diagnosis and management of neurofibromatosis type 1","volume":"103","author":"Ly KI","year":"2019","unstructured":"Ly KI, Blakeley JO. The diagnosis and management of neurofibromatosis type 1. Med Clin North Am. 2019;103(6):1035\u20131054."},{"issue":"6","key":"b8","series-title":"Curr Opin Oncol","first-page":"550","article-title":"Schwannomatosis: a realm reborn: year one","volume":"35","author":"Planet M","year":"2023","unstructured":"Planet M, Kalamarides M, Peyre M. Schwannomatosis: a realm reborn: year one. Curr Opin Oncol. 2023;35(6):550\u2013557."},{"key":"b9","series-title":"Hand (N Y)","first-page":"732","article-title":"Recurrent schwannomatosis of the hand","volume":"5","author":"Nicolescu R","year":"2020","unstructured":"Nicolescu R, Agrawal NA, Pettit RW, Netscher DT. Recurrent schwannomatosis of the hand. Hand (N Y). 2020;15(5):732-738."},{"issue":"3","key":"b10","series-title":"Clinical article. 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. Clinical article. J Neurosurg. 2011;114(3):756\u2013762."},{"issue":"10","key":"b11","series-title":"Ann Transl Med","first-page":"226","article-title":"Total en bloc resection of primary and metastatic spine tumors","volume":"7","author":"Howell EP","year":"2019","unstructured":"Howell EP, Williamson T, Karikari I,  Total en bloc resection of primary and metastatic spine tumors. Ann Transl Med. 2019;7(10):226."},{"issue":"2","key":"b12","series-title":"Pain","first-page":"149","article-title":"Clinical importance of changes in chronic pain intensity measured on an 11-point numerical pain rating scale","volume":"94","author":"Farrar JT","year":"2001","unstructured":"Farrar JT, Young JP Jr, LaMoreaux L, Werth JL, Poole MR. Clinical importance of changes in chronic pain intensity measured on an 11-point numerical pain rating scale. Pain. 2001;94(2):149\u2013158."},{"issue":"3","key":"b13","series-title":"Orthop Rev (Pavia)","first-page":"37578","article-title":"Assessment of motor function in peripheral nerve injury and recovery","volume":"14","author":"John A","year":"2022","unstructured":"John A, Rossettie S, Rafael J, Cox C, Ducic I, Mackay B. Assessment of motor function in peripheral nerve injury and recovery. Orthop Rev (Pavia). 2022;14(3):37578."},{"issue":"9","key":"b14","series-title":"Am J Med Genet A","first-page":"2672","article-title":"Understanding barriers to diagnosis in a rare, genetic disease: delays and errors in diagnosing schwannomatosis","volume":"188","author":"Merker VL","year":"2022","unstructured":"Merker VL, Slobogean B, Jordan JT,  Understanding barriers to diagnosis in a rare, genetic disease: delays and errors in diagnosing schwannomatosis. Am J Med Genet A. 2022;188(9):2672\u20132683."},{"issue":"4","key":"b15","series-title":"Neurology","first-page":"1072","article-title":"Schwannomatosis: a clinical and pathologic study","volume":"46","author":"MacCollin M","year":"1996","unstructured":"MacCollin M, Woodfin W, Kronn D, Short MP. Schwannomatosis: a clinical and pathologic study. Neurology. 1996;46(4):1072\u20131079."},{"issue":"5","key":"b16","series-title":"J Chron Dis","first-page":"373","article-title":"A new method of classifying prognostic comorbidity in longitudinal studies: development and validation","volume":"40","author":"Charlson ME","year":"1987","unstructured":"Charlson ME, Pompei P, Ales KL, MacKenzie CR. A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chron Dis. 1987;40(5):373\u2013383."},{"key":"b17","article-title":"Nerve Injuries Committee. Aids to the Examination of Peripheral Nervous System (Memorandum No. 45)","author":"Medical Research Council","year":"1976","unstructured":"Medical Research Council. Nerve Injuries Committee. Aids to the Examination of Peripheral Nervous System (Memorandum No. 45). 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Int J Mol Sci. 2021;22(11):5850."},{"issue":"2","key":"b3","series-title":"Br J Neurosurg","first-page":"171","article-title":"What to know about schwannomatosis: a literature review","volume":"36","author":"Schraepen C","year":"2022","unstructured":"Schraepen C, Donkersloot P, Duyvendak W,  What to know about schwannomatosis: a literature review. Br J Neurosurg. 2022;36(2):171\u2013174."},{"issue":"7","key":"b4","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":"10","key":"b5","series-title":"Oncologist","first-page":"1317","article-title":"Clinical features of schwannomatosis: a retrospective analysis of 87 patients","volume":"17","author":"Merker VL","year":"2012","unstructured":"Merker VL, Esparza S, Smith MJ, Stemmer-Rachamimov A, Plotkin SR. Clinical features of schwannomatosis: a retrospective analysis of 87 patients. Oncologist. 2012;17(10):1317\u20131322."},{"issue":"9","key":"b6","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":"6","key":"b7","series-title":"Med Clin North Am","first-page":"1035","article-title":"The diagnosis and management of neurofibromatosis type 1","volume":"103","author":"Ly KI","year":"2019","unstructured":"Ly KI, Blakeley JO. The diagnosis and management of neurofibromatosis type 1. Med Clin North Am. 2019;103(6):1035\u20131054."},{"issue":"6","key":"b8","series-title":"Curr Opin Oncol","first-page":"550","article-title":"Schwannomatosis: a realm reborn: year one","volume":"35","author":"Planet M","year":"2023","unstructured":"Planet M, Kalamarides M, Peyre M. Schwannomatosis: a realm reborn: year one. Curr Opin Oncol. 2023;35(6):550\u2013557."},{"key":"b9","series-title":"Hand (N Y)","first-page":"732","article-title":"Recurrent schwannomatosis of the hand","volume":"5","author":"Nicolescu R","year":"2020","unstructured":"Nicolescu R, Agrawal NA, Pettit RW, Netscher DT. Recurrent schwannomatosis of the hand. Hand (N Y). 2020;15(5):732-738."},{"issue":"3","key":"b10","series-title":"Clinical article. 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. Clinical article. J Neurosurg. 2011;114(3):756\u2013762."},{"issue":"10","key":"b11","series-title":"Ann Transl Med","first-page":"226","article-title":"Total en bloc resection of primary and metastatic spine tumors","volume":"7","author":"Howell EP","year":"2019","unstructured":"Howell EP, Williamson T, Karikari I,  Total en bloc resection of primary and metastatic spine tumors. 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