A comparative clinicopathological study of odontogenic keratocysts in basal cell nevus syndrome and control patients

We clinically and histopathologically analyzed 49 odontogenic keratocysts (OKCs), including 6 recurrent cysts. Twenty-five OKCs from 5 patients with the basal cell nevus syndrome (BCNS) were compared with 24 single OKCs (non-BCNS group). There were some differences between BCNS and non-BCNS cysts.At the time of initial diagnosis, the patients ranged in age from 14 to 73 years (mean age, 39.7 years) in the non-BCNS group, as compared with 6 to 23 years (mean age, 15.6 years) in the BCNS group. There was a slight male predominance (male-to-female ratio, 2: 1) in the non-BCNS group, but not in the BCNS group. The most common cyst sites were the mandibular molar region and the ramus of the mandible. Cysts in the maxilla were rare (13.6%) in the non-BCNS group, but not rare (38.1%) in the BCNS specimens.On the basis of the radiographic appearance of the 43 cases without recurrence, the cyst was unilocular in 59.1% of the non-BCNS group and 71.4% of the BCNS group. Six OKCs (27.3%) in the non-BCNS group were associated with an impacted tooth. In the BCNS group, 17 OKCs (81%) were associated with an impacted tooth.Enucleation followed by mechanical curettage with a round bar was performed for 38 primary cysts, and 6 recurrences were noted. Three of the 11 cysts that occurred in the maxilla were treated by enucleation by the Caldwell-Luc operation, and none of them recurred. Two multilocular extensive cysts were managed by hemimandiblectomy, and no recurrence was noted. In total, there were 6 cases of recurrent OKCs after the first operation. The recurrence rate was 9 % in the non-BCNS group and 19% in the BCNS group. The average interval until recurrence was 3 years 6 months in the non-BCNS group and 8 years in the BCNS group. Histopathologically, the presence of epithelial islands or daughter cysts in the cyst wall correlated with recurrence in BCNS cases, but not in non-BCNS cases.