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

  • Luana Eschholz Bomfin São Paulo University
  • Ana Paula M. Vivas São Paulo University
  • Andre Caroli Rocha A. C. Camargo Hospital
  • Maria Isabel W. Achatz A. C. Camargo Hospital
  • Clovis Antonio L. Pinto A. C. Camargo Hospital
  • Fabio Abreu Alves University of São Paulo; A. C. Camargo Hospital

Abstract

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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Author Biographies

Luana Eschholz Bomfin, São Paulo University
Department of Stomatology, São Paulo University, São Paulo, SP.
Ana Paula M. Vivas, São Paulo University
Department of Stomatology, São Paulo University, São Paulo, SP.
Andre Caroli Rocha, A. C. Camargo Hospital
Department of Stomatology, A. C. Camargo Hospital, São Paulo, SP.
Maria Isabel W. Achatz, A. C. Camargo Hospital
Department of Oncogenetics, A. C. Camargo Hospital, São Paulo, SP.
Clovis Antonio L. Pinto, A. C. Camargo Hospital
Department of Anatomical Pathology, A. C. Camargo Hospital, São Paulo, SP.
Fabio Abreu Alves, University of São Paulo; A. C. Camargo Hospital
Department of Stomatology, University of São Paulo, Department of Stomatology, A. C. Camargo Hospital, São Paulo, SP.
Published
2015-10-19
How to Cite
Bomfin, L. E., Vivas, A. P. M., Rocha, A. C., Achatz, M. I. W., Pinto, C. A. L., & Alves, F. A. (2015). Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study. Brazilian Journal of Oral Sciences, 12(1), 23-29. https://doi.org/10.20396/bjos.v12i1.8641129
Section
Article