Carcinomas develop, although at low frequency, in hamartomatous polyps in the Peutz-Jeghers' syndrome. With the identification of at least one of the genes involved (LKB1/STK11), attempts can be made to unravel the molecular events responsible for this hamartoma-adenoma-carcinoma sequence. Recent data indicate that this sequence is real, that the molecular pathways involved are different from those in other hereditary colorectal cancer syndromes, and that, possibly in contrast to the hamartomatous polyps in the juvenile polyposis syndrome, the genetic abnormalities occur in the epithelial compartment of the lesions.
Copyright 1999 John Wiley & Sons, Ltd.