Peutz-Jeghers syndrome: case reports and update on diagnosis and treatment.

Abstract

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.

• Authors: Ke Dong, Bo Li
• Journal: Chinese journal of digestive diseases (Chin J Dig Dis) Vol. 5 Issue 4 Pg. 160-4 ( 2004) ISSN: 1443-9611 [Print] Australia
• PMID: 15612885 (Publication Type: Case Reports, Journal Article)

Topics

• Adolescent
• Adult
• Cell Transformation, Neoplastic
• Female
• Humans
• Intestinal Neoplasms (etiology, pathology)
• Intestinal Polyps (etiology, pathology)
• Intussusception (etiology, surgery)
• Male
• Pedigree
• Peutz-Jeghers Syndrome (diagnosis, etiology, pathology)
• Recurrence