{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T21:03:21Z","timestamp":1792789401489,"version":"4.5.0"},"reference-count":111,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2021,8,10]],"date-time":"2021-08-10T00:00:00Z","timestamp":1628553600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000156","name":"Fonds de Recherche du Qu\u00e9bec - Sant\u00e9","doi-asserted-by":"publisher","award":["295641"],"award-info":[{"award-number":["295641"]}],"id":[{"id":"10.13039\/501100000156","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Association pour la Neurofibromatose du Qu\u00e9bec","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Malignant peripheral nerve sheath tumors (MPNSTs) originate from the neural crest lineage and are associated with the neurofibromatosis type I syndrome. MPNST is an unmet clinical need. In this review article, we summarize the knowledge and discuss research perspectives related to (1) the natural history of MPNST development; (2) the mouse models recapitulating the progression from precursor lesions to MPNST; (3) the role of the tumor microenvironment in MPNST development, and (4) the signaling pathways linked to MPNST development.<\/jats:p>","DOI":"10.3390\/ijms22168620","type":"journal-article","created":{"date-parts":[[2021,8,10]],"date-time":"2021-08-10T22:40:31Z","timestamp":1628635231000},"page":"8620","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development"],"prefix":"10.3390","volume":"22","author":[{"given":"Teddy","family":"Mohamad","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Functional Genomics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke, QC J1E 4K8, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camille","family":"Plante","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Functional Genomics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke, QC J1E 4K8, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5852-3507","authenticated-orcid":false,"given":"Jean-Philippe","family":"Brosseau","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Functional Genomics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke, QC J1E 4K8, Canada"},{"name":"Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Universit\u00e9 de Sherbrooke, Sherbrooke, QC J1E 4K8, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1186\/s12957-020-02036-x","article-title":"Prognosis and risk factors for malignant peripheral nerve sheath tumor: A systematic review and meta-analysis","volume":"18","author":"Cai","year":"2020","journal-title":"World J. 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