Korean J Gastroenterol.  1997 Dec;30(6):820-825.

A Case of Peutz-Jeghers Syndrome which was Misdiagnosed as Small Intestinal Adenocarcinoma

• Lee KH
• Yang DH
• Kim NY
• Choi SE
• Lim SH
• Park YJ
• Ahn KJ
• Lee GH
• Lim BC

Abstract

Peutz-Jeghers syndrome is an autosomal-dominant hereditary disorder manifested by a combina- tion of mucocutaneous pigmentations and gastrointestinal polyposis. Although the polyps are believed to he benign in nature, recently it has been realized that patients with this syndrome are subject to increasing risk for the developement of malignant neoplasm during long term follow-up. When investigating the neoplastic changes within Peutz-Jeghers polyps, pseudoinvasion-epithelial misplacement, should be carefully ruled out. In this report, we present a case of a 16-year old female who was operated or for intussusception caused by Peutz-Jeghers polyp in the small intestine, which showed pseudoinvasion-epithelial misplacement, initially misdiagnosed as adeho-carcinoma. To find out any possibility of the development of malignant neoplasm, the entire gastrointestinal tract from esophagus to rectum was searched via endoscopy and intraoperative enteroscopy, and all of the polyps were removed.

Keyword

Peutz-Jeghers syndrome; Malignant neoplasm; Pseudoinvasion; Epithelial misplacement; Intraoperative enteroscopy

MeSH Terms

Adenocarcinoma*
Adolescent
Endoscopy
Esophagus
Female
Follow-Up Studies
Gastrointestinal Tract
Humans
Intestine, Small
Intussusception
Peutz-Jeghers Syndrome*
Pigmentation
Polyps
Rectum