Peutz–Jeghers syndrome: case reports and update on diagnosis and treatment

Authors: Ke DONG; Bo LI

Source: Chinese Journal of Digestive Diseases, Volume 5, Number 4, December 2004 , pp. 160-164(5)

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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.

Keywords: Peutz–Jeghers syndrome; polyps; treatment

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1443-9573.2004.00179.x

Affiliations: 1: Department of General Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China

Publication date: 2004-12-01