가족성 선종성 용종증 수술 후 발생한 복강 내 유건종 1예
A Case of Intra-abdominal Desmoid Tumor after Total Colectomy in a Patient with Familial Adenomatous Polyposis
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초록
가족성 선종성 용종증의 치료로 전대장 절제술이 시행되지만 절제술 이후에도 여러 가지 장외 병변이 나타난다. 이러한 것으로는 문합부의 대장암 발생이나 췌장, 십이지장의 암 발생 같은 악성 종양의 발생이나 장간막, 후복막 등에 유건종의 발생 등이 있다. 이 중 유건종의 발생은 전대장 절제술을 시행한 가족성 선종성 용종증 환자의 예후를 결정하는 중요한 인자가 된다. 국내에서 가족성 선종성 용종증으로 전대장 절제술을 시행 후 복강 내 유건종의 발생에 대한 보고는 있었으나, 모두 완전 절제가 불가능하였던 예였다. 저자 등은 완전 절제가 가능하였던 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Abstract
Familial adenomatous polyposis (FAP) arises from germline mutations of the adenomatous polyposis coli (APC) gene. FAP is characterized by the occurrence of hundreds to thousands of adenomas throughout the colorectum, and there is nearly a 100% risk of colorectal cancer. In addition to polyposis coli, patients with FAP can develop a variety of extracolonic manifestations. Recent advances in screening and surgery have reduced the colon cancer occurrence and death in FAP patients, leaving desmoid tumors as a leading cause of their morbidity and mortality. Treatment of desmoid tumors is generally considered to be challenging for both the doctor and the patient. We report here on an 18 year old man with resectable intra-abdominal desmoid tumor that developed after total colectomy due to FAP and we include a review of the relevant literature. (Korean J Gastrointest Endosc 2006;33:50-53)
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