Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study

• Autor(a) principal: Bomfin, Luana Eschholz
• Data de Publicação: 2015
• Outros Autores: Vivas, Ana Paula M., Rocha, Andre Caroli, Achatz, Maria Isabel W., Pinto, Clovis Antonio L., Alves, Fabio Abreu
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Brazilian journal of oral sciences (Online)
• Texto Completo: https://periodicos.sbu.unicamp.br/ojs/index.php/bjos/article/view/8641129
• topic: Gorlin Syndrome; Keratocyst; Keratocystic Odontogenic Tumor; Nevoid Basal Cell Carcinoma syndrome; Odontologia

Aim: To assess clinicopathological features of patients with keratocystic odontogenic tumor (KCOT) associated with nevoid basal cell carcinoma syndrome (NBCCS) in a single Brazilian institution. Methods: After histopathological analyses of KCOT related to NBCCS, the medical charts of 14 patients were assessed. These patients presented a total of 31 primary and 8 recurrent KCOT. Results: Out of 14 patients, 8 presented a single KCOT, 4 showed synchronous tumors, 1 had 3 metachronous lesions and another patient had 2 synchronous lesions at initial evaluation and then developed other 3 metachronous lesions. Besides the 31 primary KCOTs, 18 lesions were located in mandible and 13 in maxilla. Most tumors presented unilocular pattern and association with a tooth. Conclusions: KCOT is a frequent manifestation of NBCCS and can be its first sign, mainly in young patients. In contrast to a previously published series, most patients presented a single lesion.