Background
Nevoid basal cell carcinoma syndrome (NBCCS), also known as basal cell nevus syndrome (BCNS), represents a series of multiorgan abnormalities known to be the consequence of abnormalities in the PTCH gene. The syndrome has been documented for 50 years, but more recent developments in molecular genetics have dramatically increased understanding of its pathophysiology and opened up molecular avenues for treatment in the future.
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