Peutz-Jeghers syndrome: case reports and update on diagnosis and treatment.
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.
Dong
Ke
K
Department of General Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China. dongke1134@hotmail.com
Li
Bo
B
D000293:Adolescent; D000328:Adult; D002471:Cell Transformation, Neoplastic; D005260:Female; D006801:Humans; D007414:Intestinal Neoplasms / Q000209:etiology / Q000473:pathology; D007417:Intestinal Polyps / Q000209:etiology* / Q000473:pathology; D007443:Intussusception / Q000209:etiology / Q000601:surgery; D008297:Male; D010375:Pedigree; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology; D012008:Recurrence