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

Abstract

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.