IN 1949, Jeghers et al1 published their classic account of the syndrome of generalized intestinal polyposis and melanin spots of the oral mucosa, lips, and digits, and wrote that "the well-known tendency for multiple polyposis of the colon to develop malignancy apparently holds to some degree for the small-bowel polyps in cases of this syndrome." This conclusion, which has practical implications favoring preventive surgery, was supported in subsequent early reports. In 1957, Bailey2 found the risk of cancer to be as high as 24%, on the basis of reviews of all 67 cases published to that time. Contrary opinions began to appear in the same year, asserting that there was no increased incidence of malignant neoplasms, and justifying surgical conservatism.
Dormandy,3 reporting on 21 patients, thought that malignant neoplasm degeneration in the small intestine was exceedingly rare if it ever occurred. Despite several histologic diagnoses of carcinoma,