Diagnosis and treatment of a rare case of adenomatoid odontogenic tumor in a young patient affected by attenuated familial adenomatosis polyposis (aFAP): case report and 5 year follow-up

M. Marrelli, A. Pacifici, G. Di Giorgio, M. Cassetta, L.V. Stefanelli, M. Gargari, L. Promenzio, S. Annibali, M.P. Cristalli, E. Chiaravalloti, L. Pacifici, M. Tatullo

Calabrodental Clinic, Oral and Maxillofacial Unit, Crotone, Italy. marco.tatullo@tecnologicasrl.com

 

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• Maxillofacial Surgery

BACKGROUND: The adenomatoid odontogenic tumor (AOT) is a quite rare odontogenic tumor, with an incidence rate of approximately 12 cases/year worldwide. Attenuated familial adenomatous polyposis (aFAP) is a syndrome characterized by a significant risk to develop colon cancer. The aim of the paper is to describe a case never reported before in the literature: an AOT developed in a patient with aFAP; moreover, we want to show how it appears 5 years after surgery and after the regeneration of the eroded bone tissue, using the Platelet-Rich Fibrin (PRF) as filling material.

CASE PRESENTATION: We report the case of a female 18 years old patient, affected by aFAP; she comes to us with a swelling on the right hemi-face. We performed several radiological exams, and they showed a neoformation approximately 2 cm in diameter: this neoformation packed the upper right canine, therefore, we hypothesized a dentigerous cyst.
We decided to proceed to open biopsy and enucleation of the lesion. An intra-operative endodontic treatment on the adjacent partially resorbed teeth was also performed. Finally, we performed a reconstruction of eroded bone tissue, by use of Platelet-Rich Fibrin as filling material.
The samples fixed and embedded in paraffin have led to the diagnosis of AOT. After 5 years from the surgery, we did not find any clear sign of relapse, in addition, the use of PRF has favored an optimal osteogenesis at the surgical site.

CONCLUSIONS: Undoubtedly, a correct diagnosis of AOT allows to have a more performing clinical and surgical approach. Furthermore, this case could document a new manifestation of aFAP in extra-intestinal site.
The onset of an AOT is quite rare in the general population, and this rarity could represent a critical point for its diagnosis; AOT onset in a patient with aFAP is a finding that could represent a new element of diagnosis and, therefore, the starting point to perform a more effective therapy.

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