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 months after the discontinuation of the study drug; * For males not to donate sperm products or semen during treatment and for 2 months after the discontinuation of the study drug; * Able and willing to swallow pill; * No malabsorption syndrome or other condition that would interfere with enteral absorption; * At least 4 weeks since last chemotherapy, investigational therapy, radiotherapy or major surgical procedure and recovered from the first study drug administration; * KCOT measures at least 1 cm in one dimension on pretreatment volumetric CT scan; * No clinically significant abnormalities with clinical laboratory assessments; Exclusion Criteria: * Concurrent anti-tumor therapy; * Completion of the most recent anti-tumor therapy (including Vismodegib) less than 4 weeks prior to the initiation of treatment (first study drug administration); * Uncontrolled medical illness; * Pregnancy or lactation; female patients who are planning to become pregnant for the duration of the study and 7 months post-treatment; * Inability or unwillingness to swallow capsules; * Any medical or psychological illness or condition preventing adequate consent; * History of significant atherosclerotic disease, including the following: * Coronary artery disease (i.e., myocardial infarction within the past year or unstable angina); * Documented carotid atheroma; * Known HIV infection; * Current alcohol abuse; * History of resistance to vismodegib (patients who previous received vismodegib for BCC and had no clinical response will be excluded).