title: Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome.
abstract: Vismodegib hedgehog signaling inhibition treatment has potential for reducing the burden of multiple skin basal cell carcinomas and jaw keratocystic odontogenic tumors. They are major criteria for the diagnosis of Gorlin syndrome, also called nevoid basal cell carcinoma syndrome. Clinical features of Gorlin syndrome are reported, and the relevance of hedgehog signaling pathway inhibition by oral vismodegib for maxillofacial surgeons is highlighted. In summary, progressed basal cell carcinoma lesions are virtually inoperable. Keratocystic odontogenic tumors have an aggressive behavior including rapid growth and extension into adjacent tissues. Interestingly, nearly complete regression of multiple Gorlin syndrome-associated keratocystic odontogenic tumors following treatment with vismodegib. Due to radio-hypersensitivity in Gorlin syndrome, avoidance of treatment by radiotherapy is strongly recommended for all affected individuals. Vismodegib can help in those instances where radiation is contra-indicated, or the lesions are inoperable. The effect of vismodegib on basal cell carcinomas was associated with a significant decrease in hedgehog-signaling and tumor proliferation. Vismodegib, a new and approved drug for the treatment of advanced basal cell carcinoma, is a specific oncogene inhibitor. It also seems to be effective for treatment of keratocystic odontogenic tumors and basal cell carcinomas in Gorlin syndrome, rendering the surgical resections less challenging.
authors.affiliation: Frankfurt Orofacial Regenerative Medicine, University Hospital Frankfurt, Frankfurt am Main, Germany.
authors.forename: Patrick
authors.initials: P
authors.lastname: Booms
authors.affiliation: Center for Radiology, University Hospital Frankfurt, Frankfurt am Main, Germany.
authors.forename: Marc
authors.initials: M
authors.lastname: Harth
authors.affiliation: Department for Oral, Cranio-Maxillofacial and Facial Plastic Surgery, University Hospital Frankfurt, Frankfurt am Main, Germany.
authors.forename: Robert
authors.initials: R
authors.lastname: Sader
authors.affiliation: Frankfurt Orofacial Regenerative Medicine, University Hospital Frankfurt, Frankfurt am Main, Germany ; Department for Oral, Cranio-Maxillofacial and Facial Plastic Surgery, University Hospital Frankfurt, Frankfurt am Main, Germany.
authors.forename: Shahram
authors.initials: S
authors.lastname: Ghanaati
keywords: Basal cell nevus syndrome; Gorlin syndrome; hedgehog-signaling inhibition; jaw keratocystic odontogenic tumors; multiple skin basal cell carcinomas; vismodegib