충수에서 발생한 포이츠 예거 증후군을 동반하지 않은 과오종성 용종 1예

A Case of Hamartomatous Polyp without Peutz-Jeghers Syndrome Arising from Appendix

  • 손위식 (분당제생병원 소화기내과) ;
  • 박주상 (분당제생병원 소화기내과) ;
  • 김지은 (분당제생병원 소화기내과) ;
  • 김봉환 (분당제생병원 소화기내과) ;
  • 유승희 (분당제생병원 소화기내과) ;
  • 한은미 (분당제생병원 해부병리과)
  • Sohn, Wee-Sik (Department of Internal Medicine, Bundang Jesaeng Hospital) ;
  • Park, Ju-Sang (Department of Internal Medicine, Bundang Jesaeng Hospital) ;
  • Kim, Ji-Eun (Department of Internal Medicine, Bundang Jesaeng Hospital) ;
  • Kim, Bong-Hwan (Department of Internal Medicine, Bundang Jesaeng Hospital) ;
  • Yoo, Seung-Hee (Department of Internal Medicine, Bundang Jesaeng Hospital) ;
  • Han, Eun-Mee (Department of Pathology, Bundang Jesaeng Hospital)
  • 투고 : 2010.04.05
  • 심사 : 2010.06.13
  • 발행 : 2010.07.30

초록

포이츠 예거 증후군은 피부점막의 색소침착과 위장관 용종증을 특징으로 하는 상염색체 우성 유전 질환으로, 점막근층에서 유래한 잘 발달된 민무늬근과 선상피가 특징인 독특한 과오종성 용종을 가진다. 포이츠 예거형 과오종성 용종이 포이츠 예거 증후군을 동반하지 않고 위장관에서 발견되는 증례도 드물게 보고된다. 지금까지 포이츠 예거 증후군을 동반하지 않은 포이츠 예거형 과오종성 용종이 충수에서 발생한 증례는 1예 뿐이었으며 외과적 수술로 제거되었다. 최근 저자들은 충수에서 발생한 포이츠 예거형 과오종성 용종을 내시경 용종절제술로 제거하였으며, 이번 증례는 내시경 치료로 제거한 충수의 포이츠 예거형 과오종성 용종의 첫 예로 문헌 고찰과 함께 보고한다.

Peutz-Jeghers syndrome is a familial syndrome consisting of mucocutaneous pigmentation and gastrointestinal polyposis and appears to be inherited as a single pleiotropic autosomal dominant gene with variable and incomplete penetrance. Cases of hamartomatous polyps of the Peutz-Jeghers type without Peutz-Jeghers syndrome have only rarely been reported. Moreover, only one case of a Peutz-Jeghers polyp at the appendix has been reported; it was resected by appendectomy. We report here on a case of a 45 year old man who had a hamartomatous polyp of the Peutz-Jeghers type arising from the appendix. The polyp was successfully removed by endoscopic polypectomy. To our knowledge, this is the first case of a hamartomatous polyp of the Peutz-Jeghers type that originated from the appendix and that was resected endoscopically.

키워드

참고문헌

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