[36] reported the results of a follow-up study of two groups of patients treated for OKC. In the first group of 52 cases, the cysts were treated conservatively by careful enucleation of the entire wall. In the second group of 40 cases, the cysts were removed by enucleation along with excision of the mucosa overlying a perforation of the cortical bone, which was determined at operation. Before considering removal, all cysts in this group were treated with Carnoy’s solution. The recurrence rate in their first group was 13.5% in a 1-21 year follow-up while the recurrence rate in their second group was 2.5% in a 1-10 year follow-up. Furthermore, the current studies show similar results [Table 1] regarding the frequent recurrence of the keratocyst in NBCCS. Furthermore, all of those studies describing cohort, case series and miscellaneous clinical reports.

No randomized controlled trials for the treatment of keratocyst in NBCCS were located in the literature. Table 1 A description of odontogenic keratocyst in nevoid basal cell carcinoma syndrome from clinical view (2010-2012) DISCUSSION The OKC represent from 65% to 75% of the cases of the NBCCS.[52] These cysts represent a particular entity that has been of interest, mainly due to biological aggressiveness and to the great amount of recurrence.[1,7] Recently and based on the intrinsic growth potential of its epithelial coating, they have been re-classified and called OKC tumors and they have been included in the odontogenic neoplasias.

[7,11,26] The keratocysts have a well-defined scale-like parakeratinized stratified epithelium with an average thickness of 5-8 cells, with a basal layer in which cells present themselves fenced up in a corrugated surface and a connective wall rich in mucopolisacarids, without inflammatory infiltration and with a variable number of microcysts and epithelial islets.[9,50] Its high potential of recurrence is justified by the high mitotic epithelial activity, the frequency of satellite cysts, pieces of epithelium and prolific dental sheet and by the existence of a epithelial coating thicker than in other jaw cysts.[7,10,11] The treatment modalities for the keratocysts vary from simple enucleation with curettage, to the enucleation with peripheral osteotomy or to osseous resection in block. This last technique is the most aggressive and it logically follows that the recurrence rate decreases.

[61] There are also more conservative options such as the local parietal therapy with Carnoy solution, with cryotherapy or marsupialization of the cysts, or decompression followed by a secondary enucleation.[8] Nevertheless, those methods are not efficient in the long-term and their use is considered to be controversial. It is believed that the nature of the treatment of keratocyst is depending on the following factors: Lesion size, lesion extension, location, possible AV-951 cortical and soft parts damage, the age and whether it is a primary or recurrent lesion.