DOI QR코드

DOI QR Code

A Case of Multiple Tumors Located in Structures Other than the Colon in a Patient with Familial Adenomatous Polyposis

대장 외에 다발성 종양이 발생한 가족성샘종폴립증 1예

  • Doo, Eun Young (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Kim, Dong Chan (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Jung, Hye Mi (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Yoon, Jun Sik (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Kim, Jeong Eun (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Choi, Kyung Soo (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Choi, Ho Soon (Department of Internal Medicine, Hanyang University College of Medicine)
  • 두은영 (한양대학교 의과대학 내과학교실) ;
  • 김동찬 (한양대학교 의과대학 내과학교실) ;
  • 정혜미 (한양대학교 의과대학 내과학교실) ;
  • 윤준식 (한양대학교 의과대학 내과학교실) ;
  • 김정은 (한양대학교 의과대학 내과학교실) ;
  • 최경수 (한양대학교 의과대학 내과학교실) ;
  • 최호순 (한양대학교 의과대학 내과학교실)
  • Received : 2012.03.30
  • Accepted : 2012.08.16
  • Published : 2013.04.01

Abstract

Most patients with familial adenomatous polyposis (FAP) undergo prophylactic colectomy. As a result, extracolonic manifestations including benign and malignant tumors are more common in patients with FAP, and duodenal cancer is now the main cause of death. However, duodenal adenomas and carcinomas are detected less frequently in Korea and Japan than in Western countries, and gastric cancer is more prevalent than in Western countries. In the present case, a 63-year-old man, who had undergone a total colectomy and an ileostomy 14 years previously, presented at our outpatient clinic complaining of discomfort at the ileostomy site. The patient had adenomas in the gallbladder and ileum, and adenocarcinomas in the duodenum and stomach. The interval between development of duodenal cancer and stomach cancer was only 4 months. Therefore, we suggest that more frequent endoscopic surveillance may be needed for these cases.

저자들은 가족성샘종폴립증 환자가 대장 외 증상으로 회장과 담낭에 용종이 있었고, 짧은 간격의 추적 상부위장관내시경 검사를 시행했음에도 불구하고 십이지장 유두부 선암종과 위의 바닥샘종과 연관된 위 선암종이 발생한 증례를 경험하였다. 과거와 비교하여 가족성샘종폴립증 환자들이 선암종이 발생하기 전에 예방적으로 대장 절제술을 받는 경우가 증가하면서 대장 외 증상으로 인한 사망률이 점차 증가하고 있다. 내시경과 초음파를 이용한 주기적인 추적검사가 필요할 것으로 생각한다.

Keywords

References

  1. Anaya DA, Chang GJ, Rodriguez-Bigas MA. Extracolonic manifestations of hereditary colorectal cancer syndromes. Clin Colon Rectal Surg 2008;21:263-272. https://doi.org/10.1055/s-0028-1089941
  2. Gallagher MC, Phillips RK, Bulow S. Surveillance and management of upper gastrointestinal disease in familial adenomatous polyposis. Fam Cancer 2006;5:263-273. https://doi.org/10.1007/s10689-005-5668-0
  3. Park SY, Ryu JK, Park JH, et al. Prevalence of gastric and duodenal polyps and risk factors for duodenal neoplasm in korean patients with familial adenomatous polyposis. Gut Liver 2011;5:46-51. https://doi.org/10.5009/gnl.2011.5.1.46
  4. Hofgartner WT, Thorp M, Ramus MW, et al. Gastric adenocarcinoma associated with fundic gland polyps in a patient with attenuated familial adenomatous polyposis. Am J Gastroenterol 1999;94:2275-2281. https://doi.org/10.1111/j.1572-0241.1999.01312.x
  5. Park JG, Kim IJ. Hereditary colorectal cancer. Korean J Gastroenterol 2005;45:78-87.
  6. Vasen HF, Moslein G, Alonso A, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008;57:704-713. https://doi.org/10.1136/gut.2007.136127
  7. Spigelman AD, Talbot IC, Penna C, et al. Evidence for adenoma-carcinoma sequence in the duodenum of patients with familial adenomatous polyposis: the Leeds Castle Polyposis Group (Upper Gastrointestinal Committee). J Clin Pathol 1994;47:709-710. https://doi.org/10.1136/jcp.47.8.709
  8. Abraham SC, Nobukawa B, Giardiello FM, Hamilton SR, Wu TT. Fundic gland polyps in familial adenomatous polyposis: neoplasms with frequent somatic adenomatous polyposis coli gene alterations. Am J Pathol 2000;157:747-754. https://doi.org/10.1016/S0002-9440(10)64588-9
  9. Komorowski RA, Tresp MG, Wilson SD. Pancreaticobiliary involvement in familial polyposis coli/Gardner's syndrome. Dis Colon Rectum 1986;29:55-58. https://doi.org/10.1007/BF02555290
  10. Willson SA, Princenthal RA, Law B, Leopold GR. Gallbladder carcinoma in association with polyposis coli. Br J Radiol 1987;60:771-773. https://doi.org/10.1259/0007-1285-60-716-771