INTESTINE Vol.9 No.6(2-2-1)

特集名 大腸ポリポーシスと関連疾患 (遺伝性大腸癌)
題名 ポリポーシス (2) Peutz-Jeghers症候群 a. 総説
発刊年月 2005年 11月
著者 石田 秀行 埼玉医科大学総合医療センター消化管・一般外科
著者 岩間 毅夫 佐々木研究所付属杏雲堂病院外科
【 要旨 】 要旨はありません。
Theme Polyposis of the colon and rectum, and other related conditions : hereditary colorectal cancer
Title Peutz -Jeghers syndrome
Author Hideyuki Ishida Department of Digestive Tract and General Surgery, Saitama Medical Center / School
Author Takeo Iwama Department of Surgery, Kyoundo Hospital, Sasaki Foundation
[ Summary ] Peutz-Jeghers syndrome (PJS) is an autosomal dominantly inherited disorder which is characterized by mucocutaneous melanin pigmentation and multiple hamartomous polyps in the gastrointestinal tract. The LKB1 / STK11 gene at chromosome 19p13.3 has recently been determined to be a causative gene for PJS.
Clinically, removing polyps in the gastrointestinal tract is important because these polyps can cause bleeding or intusussception, and can also develop into cancer. The methods for removing small-bowel polyps include various options such as intestinal eversion methods, multiple enterotomy guided by palpation, and intraoperative endoscopic treatment. As a modification of conventional intraoperative endoscopy, we developed an intestinal pleating technique, going over the corrugated anesthetic tube inserted through an enterotomy site. Our method has the following merits ; (1) it is feasible even in emergency cases, (2) it prevents contamination of the surgical field, and (3) it facilitates the easier collection of polypectomized lesions.
Surveillance for neoplasms is also important in the clinical management of patients with JPS, because these patients are at high risk of developing cancers in the gastrointestinal tract, uterus, ovary, breast, and other organs.