Gardner’s Syndrome and Myxoma: A Rare Clinical Case with Very Early Onset

Author Info

Juan Pablo Rodríguez Arias Elena Gómez García Marta Maria Pampin Martinez Jose Luis Cebrian Carretero

Corresponding Author

Juan Pablo Rodríguez Arias
Department of Children’s Oral and Maxillofacial, La Paz Hospital, Madrid, Spain

A B S T R A C T

Background: Gardner’s syndrome (GS) is an autosomal dominant inherited disease that predisposes individuals to a high risk of developing colonic polyposis, colorectal cancers, and multiple maxillofacial hard and soft tissue tumors. Odontogenic myxoma (OM) is classified as a benign locally invasive tumor of the jaws. It is estimated as the third most frequent odontogenic tumor, between 2% and 5% of cases. In the present study, we report and discuss a case of odontogenic myxoma in a 13-month-old patient diagnosed with GS. Case Presentation: A 13-month-old patient was referred to our department with a fast-growing tumor located in the left nasomaxillary region and a family history of GS. Computed tomography imaging of the face with contrast was performed, showing a well-defined, insufflating osteolytic lesion located in the left nasomaxillary region, extending up to the inferior orbital wall. The tumor was biopsied, and he was diagnosed with OM. Radical resection was performed and the anterior maxilla wall was reconstructed with PDS. Subsequently, germline mutation in APC gene was found in the patient. Follow up was carried out up to seven years after the intervention, an excellent cosmetic outcome and no signs of recurrence were observed in the area. Conclusion: Oral and maxillofacial surgeons must be aware of the different lesions which may be associated with systemic syndromes. OM may be first manifestation related to early diagnosis of GS and, lastly, colon cancer. OM could be an indication of referring to genetic counselling to determine the presence of the mutation in patients with or without familiar history.

Article Info

Article Type

Case Report

Publication history

Received: Wed 30, Mar 2022
Accepted: Fri 15, Apr 2022
Published: Fri 29, Apr 2022

Copyright

© 2021 Juan Pablo Rodríguez Arias. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository.
DOI: 10.31487/j.SCR.2022.04.02