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2015 ; 5
(1
): 14-9
Nephropedia Template TP
gab.com Text
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English Wikipedia
Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas
as well as keratocystic odontogenic tumors in Gorlin syndrome
#MMPMID26389028
Booms P
; Harth M
; Sader R
; Ghanaati S
Ann Maxillofac Surg
2015[Jan]; 5
(1
): 14-9
PMID26389028
show ga
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.