OBJECTIVE: To analyze the etiology and diagnostic methods of Peutz-Jeghers syndrome (PJS) and thus establish a treatment strategy. METHODS: Clinical data from six patients with PJS were evaluated from the aspect of familial history, carcinogenesis and recurrence of polyps. RESULTS: The fathers of four and the mother of one of the six patients had PJS. The grandfather of three of the six patients had PJS. There was a history of cancer in three of the five families. Case 4 underwent two laparotomies for intussusceptions caused by recurrent polyps of the small intestine. Case 5 also had recurrent small intestinal polyps and required a laparotomy after 1 year of initial treatment. Polyps in cases 1 and 4 showed adenomatous changes and those in case 2 were associated with gastric cancer. CONCLUSIONS: Patients with PJS have a strong family history of cancer and a high incidence of recurrence of small intestinal polyps. Malignant changes of polyps may follow the hamartoma-adenoma-carcinoma sequence. Careful follow-up is mandatory for gastrointestinal tract symptoms, and other solid organs that are susceptible to malignant change.

Digestive tract hemorrhage and recurrent ileus. Peutz-Jeghers syndrome-case report.Wiad Lek. 2003;56(3-4):192-8.

Authors describe a case of 24 years old woman with recurrent lower GI bleeding and mechanical obstruction. Crohn's disease was diagnosed at the beginning. It was impossible to treat the patient's profound anemia with blood transfusions due to her religion believes. The diagnosis of Peutz-Jeghers syndrome was made postoperatively. During operation mechanical obstruction (invagination of terminal ileum into ascending colon) was released. The invagination was caused by 3 cm large polyp localized in terminal ileum. Subsequently, 8 polyps (varying in size between 0.5 and 3 cm) in the small intestine were localized using intraoperative upper GI endoscopy and enteroscopy and finally surgically removed. Typical hamartomas were found on histopathological examination of removed polyps. Authors present modern attitude to Peutz-Jeghers syndrome--its diagnostics, treatment and follow-up.

A solitary Peutz-Jeghers type hamartomatous polyp in the duodenum--a case report.Jpn J Surg. 1988 Jul;18(4):475-7.

A 56-yr-old woman with a solitary hamartomatous polyp confined to within the third portion of the duodenum, underwent a successful endoscopic polypectomy. The histological findings were identical to Peutz-Jeghers polyp but all the other clinical features of Peutz-Jeghers syndrome were absent.