Basal cell carcinomas: attack of the hedgehog.
Basal cell carcinomas (BCCs) were essentially a molecular 'black box' until some 12 years ago, when identification of a genetic flaw in a rare subset of patients who have a great propensity to develop BCCs pointed to aberrant Hedgehog signalling as the pivotal defect leading to formation of these tumours. This discovery has facilitated a remarkable increase in our understanding of BCC carcinogenesis and has highlighted the carcinogenic role of this developmental pathway when aberrantly activated in adulthood. Importantly, a phase 1 first-in-human trial of a Hedgehog inhibitor has shown real progress in halting and even reversing the growth of these tumours.
Epstein
Ervin H
EH
Children's Hospital Oakland Research Institute, 5700 Martin Luther King Jr Way, Oakland, California 94609, USA. eepstein@chori.org
D000818:Animals; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001478:Basal Cell Nevus Syndrome / Q000235:genetics / Q000503:physiopathology; D002280:Basal Cell Carcinoma / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism*; D020022:Genetic Predisposition to Disease; D053823:Hedgehog Proteins / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D051379:Mice; D000072079:Patched Receptors; D011956:Receptors, Cell Surface / Q000235:genetics; D015398:Signal Transduction / Q000187:drug effects; D012878:Skin Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism*; D014481:United States / Q000453:epidemiology
D000970:Antineoplastic Agents; D053823:Hedgehog Proteins; D000072079:Patched Receptors; D011956:Receptors, Cell Surface