We have managed four cases of
Peutz-Jeghers syndrome (PJS) in children. Fathers of three of these patients had PJS. There was also a family history of
cancer in three cases, with
pancreatic cancer in a father, colonic and
laryngeal cancers in a grandfather, and hepatic and
gastric cancers in a grandmother. It is presumed that in each of the cases, the largest
polyp was responsible for initial symptoms. Preoperative examination revealed additional small
polyps in the whole alimentary tract except for the oesophagus. Patients underwent
laparotomy to remove the largest
polyps and subsequent intraoperative endoscopic polypectomy for other small
polyps, to minimize intestinal resection. Follow-up gastrointestinal examinations, including upper gastrointestinal series, small intestinal contrast study, and barium enema, were repeated about once a year. Three of four cases showed recurrent small
intestinal polyps, and one required a second
laparotomy because of recurrent
abdominal pain. In conclusion, patients with PJS occurring in childhood have a strong hereditary family history of
cancer and a high incidence of recurrence. Careful follow-up examination is mandatory for the gastrointestinal tract, as well as other solid organs that are susceptible to malignant change, throughout a patient's life.