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

Brazilian Journal of Oral Sciences

Luana Eschholz Bomfin²  Ana Paula M. Vivas²  Andre Caroli Rocha¹  Maria Isabel W. Achatz¹  Clovis Antonio L. Pinto¹  Fabio Abreu Alves¹ 

[1] ,São Paulo University Department of Stomatology São Paulo SP ,Brazil

关键词: Gorlin syndrome;    keratocyst;    keratocystic odontogenic tumor;    nevoid basal cell carcinoma syndrome;   

DOI  :  10.1590/S1677-32252013000100006

来源: SciELO

【 摘 要 】

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.

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