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Searches in Embase, PubMed, Web of Science and Scopus were conducted for the identification of case reports\/case series in English language. The risk of bias was assessed using the Joanna Briggs Institute tool. Outcomes were evaluated by Cox regression and Kaplan\u2013Meier methods. A total of 306 articles were retrieved, 50 of which reporting 57\u00a0MPNST were included. The lesion showed a predilection for the mandible (\n                    <jats:italic>n<\/jats:italic>\n                    \u00a0=\u00a018\/31.57%) of middle\u2010aged adults (~40.5\u00a0years) with a male\/female ratio of 1.1:1. The individuals were mostly symptomatic with a mean evolution time of 9.6\u00a0months. Surgical removal plus adjuvant therapy (especially radiotherapy) was the main approach (51.86%). Recurrence was reported in 39.62% of cases. Nodal and distant metastases were identified in 28.26% and 26.66% of cases, respectively. The 2\u2010year cumulative survival rate was 55%. Independent predictors of poor survival were the presence of neurofibromatosis type 1 (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.04) and distant metastases (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.004). The diagnosis of MPNST is challenging due to the variety of its clinical and histopathological presentations. Local aggressiveness and the potential for metastases are common outcomes of this neoplasm.\n                  <\/jats:p>","DOI":"10.1111\/odi.13982","type":"journal-article","created":{"date-parts":[[2021,8,1]],"date-time":"2021-08-01T13:43:17Z","timestamp":1627825397000},"page":"2072-2082","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Malignant peripheral nerve sheath tumour of the oral and maxillofacial region\u2014A systematic review"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0993-936X","authenticated-orcid":false,"given":"Lauren Frenzel","family":"Schuch","sequence":"first","affiliation":[{"name":"Department of Oral Diagnosis Piracicaba Dental School Universidade Estadual de Campinas  Piracicaba Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura Borges","family":"Kirschnick","sequence":"additional","affiliation":[{"name":"Department of Oral Diagnosis Piracicaba Dental School Universidade Estadual de Campinas  Piracicaba Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6599-3950","authenticated-orcid":false,"given":"Jos\u00e9 Alcides Almeida","family":"de Arruda","sequence":"additional","affiliation":[{"name":"Department of Oral Surgery, Pathology and Clinical Dentistry School of Dentistry Universidade Federal de Minas Gerais  Belo Horizonte Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isadora Peres","family":"Klein","sequence":"additional","affiliation":[{"name":"Department of Oral Pathology School of Dentistry Universidade Federal do Rio Grande do Sul  Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9834-5194","authenticated-orcid":false,"given":"Felipe Martins","family":"Silveira","sequence":"additional","affiliation":[{"name":"Molecular Pathology Area School of Dentistry Universidad de la Rep\u00fablica (UDELAR)  Montevideo Uruguay"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6560-9536","authenticated-orcid":false,"given":"Ana Carolina Uchoa","family":"Vasconcelos","sequence":"additional","affiliation":[{"name":"Diagnostic Centre for Oral Diseases School of Dentistry Universidade Federal de Pelotas  Pelotas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2040-6617","authenticated-orcid":false,"given":"Alan Roger","family":"Santos\u2010Silva","sequence":"additional","affiliation":[{"name":"Department of Oral Diagnosis Piracicaba Dental School Universidade Estadual de Campinas  Piracicaba Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1rcio Ajudarte","family":"Lopes","sequence":"additional","affiliation":[{"name":"Department of Oral Diagnosis Piracicaba Dental School Universidade Estadual de Campinas  Piracicaba Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1624-0178","authenticated-orcid":false,"given":"Vinicius Coelho","family":"Carrard","sequence":"additional","affiliation":[{"name":"Department of Oral Pathology School of Dentistry Universidade Federal do Rio Grande do Sul; Department of Oral Medicine Hospital de Cl\u00ednicas de Porto Alegre Universidade Federal do Rio Grande do Sul  Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pablo Agustin","family":"Vargas","sequence":"additional","affiliation":[{"name":"Department of Oral Diagnosis Piracicaba Dental School Universidade Estadual de Campinas  Piracicaba Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco Antonio Trevizani","family":"Martins","sequence":"additional","affiliation":[{"name":"Department of Oral Pathology School of Dentistry Universidade Federal do Rio Grande do Sul; Department of Oral Medicine Hospital de Cl\u00ednicas de Porto Alegre Universidade Federal do Rio Grande do Sul  Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1447-2135","authenticated-orcid":false,"given":"Vivian Petersen","family":"Wagner","sequence":"additional","affiliation":[{"name":"Academic Unit of Oral and Maxillofacial Medicine and Pathology Department of Clinical Dentistry University of Sheffield  Sheffield UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8662-5965","authenticated-orcid":false,"given":"Manoela Domingues","family":"Martins","sequence":"additional","affiliation":[{"name":"Department of Oral Diagnosis Piracicaba Dental School Universidade Estadual de Campinas  Piracicaba Brazil"},{"name":"Department of Oral Pathology School of Dentistry Universidade Federal do Rio Grande do Sul  Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,8,12]]},"reference":[{"issue":"7","key":"e_1_2_9_2_1","first-page":"420","article-title":"Malignant peripheral nerve sheath tumour of maxilla","volume":"21","author":"Ali N. 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