Peutz-Jeghers syndrome with intestinal carcinoma: report of the association in one family.

ABSTRACT

The Peutz-Jeghers Syndrome is a rare, genectically inherited disorder. There are insufficient data to establish this syndrome as a precancerous condition, but documented cases of gastrointestinal cancer arising in this syndrome have been reported with an incidence of 2--3%. Since the syndrome is transmitted as an autosomal dominant and is associated with cancer, family members so afflicted may show an increased cancer risk. This is the first reported case of a father and son with both Peutz-Jeghers Syndrome and colonic carcinoma.