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Brazilian Journal of Oral Sciences
Piracicaba Dental School - UNICAMP
EISSN: 1677-3225
Vol. 12, No. 1, 2013, pp. 23-29
Bioline Code: os13006
Full paper language: English
Document type: Research Article
Document available free of charge

Brazilian Journal of Oral Sciences, Vol. 12, No. 1, 2013, pp. 23-29

 en Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study
Bomfin, Luana Eschholz; Vivas, Ana Paula M.; Rocha, Andre Caroli; Achatz, Maria Isabel W.; Pinto, Clovis Antonio L. & Alves, Fabio Abreu


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.

Gorlin syndrome; keratocyst; keratocystic odontogenic tumor; nevoid basal cell carcinoma syndrome

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