Peutz–Jeghers syndrome: case reports and update on diagnosis and treatment
Authors: Ke DONG1; Bo LI
Source: Chinese Journal of Digestive Diseases, Volume 5, Number 4, December 2004 , pp. 160-164(5)
Publisher: Blackwell Publishing
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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: 10.1111/j.1443-9573.2004.00179.x
Affiliations: 1: Department of General Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
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