Presentation of a keratocystic odontogenic tumor with agenesis: a case report

INTRODUCTION: We analyzed the etiopathogenetic, clinical, radiographic, and histopathologic aspects of keratocystic odontogenic tumors, particularly in association with dental anomalies of number, with the aim of providing useful information for their correct diagnosis, treatment, and prognosis within a multidisciplinary approach. CASE PRESENTATION: A 14-year-old Caucasian girl presented for observation of bilateral agenesis of the upper incisors, which was diagnosed by orthopantomography. Approximately one year after starting orthodontic treatment, the patient went to the emergency department because of a phlegmonous tumefaction of the lateroposterior upper left maxillary region. Diagnostic orthopantomography and axial computed tomography scan results of the facial skeleton revealed a large lesion occupying the left maxillary sinus, rhizolysis of dental elements 26 and 27, and dislocation of dental element 28. The lesion and infected sinus mucosa were removed through surgical antral-cystectomy with the Caldwell-Luc approach. Histological examination of the lesion confirmed the suspected diagnosis of keratocystic odontogenic tumor. The 12-month follow-up orthopantomography and computed tomography scan results showed good trabecular bone formation in the lesion area. The 24-month follow-up results showed optimal healing in the area of the lesion, positive pulp vitality tests for teeth 26 and 27, and good periodontal tissue healing, as verified through periodontal probing. CONCLUSIONS: Combined with our observations from a careful review of the literature, the results of the case study suggest that keratocystic odontogenic tumor and dental agenesis probably do not develop through a common genetic cause. More likely, they are caused by related environmental factors. Management of this case required the multidisciplinary collaboration of different specializations and careful planning to devise a correct therapeutic protocol and reach a favorable prognosis.