Clinical, radiographic, pathological and inherited characteristics of odontogenic keratocyst in nevoid basal cell carcinoma syndrome: a study in three Chilean families.

INTRODUCTION: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant condition characterized by the development of odontogenic keratocyst (OKC), basal cell carcinomas and palmar-plantar pits among other conditions. Reports about Latin American population are scarce. OBJECTIVE: To analyze the clinical, radiographic, histopathologic and inherited features of odontogenic keratocyst and palmar pits in three Chilean families with nevoid basal cell carcinoma syndrome. MATERIAL AND METHODS: After histopathologic diagnosis of OKC, notified consent was requested and evaluation of the affected patients and their families was done. RESULTS: Two families appeared to have only one affected adolescent, and both of them were considered de novo cases. In the third family, three affected members participated in this study, with an autosomal dominant presentation. All affected patients had OKC and palmar pits. Basal cell carcinomas were present only among adult patients. All examined patients were from Latin American ethnic groups. CONCLUSIONS: Patients with NBCCS had single or multiple OKCs that were located more frequently in the mandibular area. One family had autosomal dominant inheritance and the other two families were de novo cases. None of the three teenage patients had basal cell carcinomas.

Assessment of second (I2M) and third (I3M) molar indices for establishing 14 and 16 legal ages and validation of the Cameriere's I3M cut-off for 18 years old in Chilean population.

This retrospective cross-sectional study has two-fold aims: the first is to assess new cut-offs at the legal age thresholds (LATs) of 14 and 16 years old and the second is to validate the cut-off of third molar index I3M=0.08 for 18 years of age in Chilean people. Orthopantomographs from 822 Chilean children aged from 11 to 22 (472 girls and 350 boys) were analysed. For LAT of 14 years, cut-offs were found using the ROC curves singly for boys and girls. The cut-offs for boys were I2M=0.16 and I3M=0.73 while for girls we obtained I2M=0.10 and I3M=0.77. For LAT of 16 years we obtained the same cut-offs regardless of gender, which were 0.06 and 0.36 for I2M and I3M respectively. Concerning the validity of I3M cut-off for 18 years old in Chilean population, the proportion of correctly classified individuals was 83% and estimated post-test probability, PPV, was 93.2%, with a 95% confidence interval equals to 91.3%, 94.6%. Hence, the probability that a subject positive on the test was 18 years of age or older was 93.2%, confirming the validation of the I3M cut-off for Chilean population.