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World J Exp Med. Dec 20, 2024; 14(4): 98005
Published online Dec 20, 2024. doi: 10.5493/wjem.v14.i4.98005
Clinicopathological and molecular insights into odontogenic tumors associated with syndromes: A comprehensive review
Lauren Frenzel Schuch, Felipe Martins Silveira, Vanesa Pereira-Prado, Estefania Sicco, Deepak Pandiar, Mariana Villarroel-Dorrego, Ronell Bologna-Molina
Lauren Frenzel Schuch, Felipe Martins Silveira, Vanesa Pereira-Prado, Estefania Sicco, Ronell Bologna-Molina, Department of Diagnosis in Pathology and Oral Medicine, Faculty of Dentistry, Universidad de la República, Montevideo 1600, Uruguay
Deepak Pandiar, Department of Oral Pathology and Microbiology, Saveetha Dental College and Hospitals Chennai, Saveetha Institute of Medical and Technical Sciences, Saveetha University, Chennai, Chennai 600077, Tamil Nādu, India
Mariana Villarroel-Dorrego, Department of Oral Pathology, Oral Medicine, School of Dentistry, Universidad Central de Venezuela, Venezuela, Caracas 1051, Distrito Capital, Venezuela
Co-first authors: Lauren Frenzel Schuch and Ronell Bologna-Molina.
Author contributions: Schuch LF and Bologna-Molina R designed and performed research, and wrote the paper; Silveira FM, Pereira-Prado V, Sicco E, and Pandiar D revised the article; Villarroel-Dorrego M contributed with the representative cases.
Conflict-of-interest statement: All the authors declare that they have no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ronell Bologna-Molina, DDS, MSc, PhD, Professor, Molecular Pathology Area, Faculty of Dentistry, University of the Republic, Montevideo 11600, Uruguay. ronellbologna@hotmail.com
Received: June 14, 2024
Revised: August 19, 2024
Accepted: August 26, 2024
Published online: December 20, 2024
Processing time: 138 Days and 10.6 Hours
Abstract

The association between genetic syndromes and odontogenic tumors encompasses several entities, reflecting the intricate interplay between genetic factors and the development of these lesions. The present study aimed to comprehensively investigate the associations between genetic syndromes and odontogenic tumors. We delineated the diverse spectrum of syndromic connections, including key syndromes such as Gardner syndrome, Gorlin syndrome, Schimmelpenning syndrome, and others. Our findings underscore the clinical significance of recognizing odontogenic tumors associated with genetic syndromes as diagnostic indicators for early intervention. We advocate for multidisciplinary collaboration among clinicians, geneticists, and researchers to deepen our understanding of the underlying mechanisms driving these syndromic associations. In light of this, our study contributes to the growing body of knowledge in dentistry and medical genetics, offering insights that may inform clinical practice and enhance patient care for individuals affected by genetic syndromes and odontogenic tumors.

Keywords: Genetic syndrome; Odontogenic tumors; Head and neck tumors; Misdiagnosis; Genetic mutations

Core Tip: It is important for the health professional to know that some odontogenic tumors have a close relationship with some genetic syndromes. Knowledge of this relationship can help a correct diagnosis and comprehensive treatment of the patient. Thus, the aim of the present review was to comprehensively investigate the associations between genetic syndromes and odontogenic tumors.