A Phase 2, Single-center, Single-arm, Open Label Trial of Vismodegib in Patients With Keratocystic Odontogenic Tumors

Inclusion Criteria: * Males and females, 18 years of age and above at the time the informed consent form is signed; * Able to understand and sign the Informed Consent Form and other necessary paperwork prior to initiation of study procedures; * Able to communicate with the investigator/study site personnel, understand and comply with the study requirements, and willing to return for specified visits at the appointed time; * Patients who have received prior treatment for their KCOT and with a diagnosis of recurrent (maxillary or mandibular) sporadic KCOT or NBCCS-associated KCOT (single or multiple); * Diagnosis of KCOT will be done by past pathology report or by biopsy at the study site, if applicable; * Willingness to consent to biopsy of the lesion, if needed; * Willingness to delay excision of the target tumor site, unless evidence of disease progression or lack of drug tolerability; * Willingness to donate blood for genetic testing; * For female patients of childbearing potential, agreement to use two acceptable methods of birth control, including one barrier method during the study and 7 months after discontinuation of study drug; * For males with female partners of childbearing potential, agreement to use a male condom (with spermicide) and to advise their female partners to use an acceptable method of birth control during the study and for 2 months after the discontinuation of the study drug; * Agreement not to donate blood/blood products during the study and for 7 mo…