{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T20:32:20Z","timestamp":1777062740751,"version":"3.51.4"},"reference-count":43,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2024,9,2]],"date-time":"2024-09-02T00:00:00Z","timestamp":1725235200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Spanish grants from MICINN","award":["PID2020-115371RB-I00"],"award-info":[{"award-number":["PID2020-115371RB-I00"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>A squamous odontogenic tumor (SOT) is an epithelial locally benign neoplasia derived from the periodontium of the jaws. It is considered a lesion of low incidence. Predominantly, it affects the mandible, although both jaw bones may be involved. Here, we discuss the malignant clinical evolution of an SOT lesion in an 80-year-old female patient. The patient exhibited an expansive triangular lesion at the inferior right quadrant. Surgery was performed and an SOT was diagnosed (2019). Two years after, the lesion grew, and the analysis of the biopsy revealed SOT malignization with pleomorphic atypical squamous cells, characteristics of a squamous cell carcinoma (2021). Massive DNA sequencing of formalin-fixed\u2013paraffin-embedded specimens of the initial and relapsed tumors indicated pathogenic mutations in RET and POLE genes in both tumors, loss of ALK, and gain of CDKN1B and MAP2K in the relapse. In addition, the clinical, radiographic, and microscopic features of this neoplasm are discussed and compared with those already published. The case presented contributes to the better understanding of this SOT tumor entity and to indicates its malignant evolution, together with its biological behavior and its histologic, clinical, and radiographic features. Also, it aims to stress the importance of deeper genetic analyses in rare diseases to uncover mutations that help to select a personalized treatment.<\/jats:p>","DOI":"10.3390\/ijms25179547","type":"journal-article","created":{"date-parts":[[2024,9,3]],"date-time":"2024-09-03T12:22:33Z","timestamp":1725366153000},"page":"9547","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Exceptional Evolution of a Squamous Odontogenic Tumor in the Jaw: Molecular Approach"],"prefix":"10.3390","volume":"25","author":[{"given":"Miguel","family":"Alonso-Juarranz","sequence":"first","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Department of Surgery, Faculty of Medicine, Universidad Complutense, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oscar De La","family":"Sen","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Department of Surgery, Faculty of Medicine, Universidad Complutense, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pablo","family":"P\u00e9rez","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Histopatology Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Aranzazu","family":"Gonz\u00e1lez-Corch\u00f3n","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Department of Surgery, Faculty of Medicine, Universidad Complutense, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Santiago","family":"Cabezas-Camarero","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Oncology Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melchor","family":"Saiz-Pardo","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Histopatology Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jesus","family":"Vi\u00f1as-Lopez","sequence":"additional","affiliation":[{"name":"Secugen, Center for Biological, Research Margarita Salas, CSIC, 28040 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucia","family":"Recio-Poveda","sequence":"additional","affiliation":[{"name":"Department of Molecular Biomedicine, Center for Biological, Research Margarita Salas, CSIC, 28040 Madrid, Spain"},{"name":"Rare Diseases Networking Biomedical Research Centre (CIBERER), 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6310-2245","authenticated-orcid":false,"given":"Luisa Mar\u00eda","family":"Botella","sequence":"additional","affiliation":[{"name":"Department of Molecular Biomedicine, Center for Biological, Research Margarita Salas, CSIC, 28040 Madrid, Spain"},{"name":"Rare Diseases Networking Biomedical Research Centre (CIBERER), 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Farzin","family":"Falahat","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery Service, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Department of Surgery, Faculty of Medicine, Universidad Complutense, 28040 Madrid, Spain"},{"name":"Rare Diseases Networking Biomedical Research Centre (CIBERER), 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.humpath.2017.05.014","article-title":"The Fourth Edition of the Head and Neck World Health Organization Blue Book: Editors\u2019 Perspectives","volume":"66","author":"Chan","year":"2017","journal-title":"Hum. 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