Surgical Management in Lynch Syndrome

  • Raul Cutait
  • Miguel A. Rodriguez-BigasEmail author
Part of the M.D. Anderson Solid Tumor Oncology Series book series (MDA, volume 5)


Surgery in the Lynch Syndrome can be curative, palliative, and occasionally prophylactic or preventive. An abdominal colectomy and ileorectal anastomosis is the usual procedure recommended in a patient with newly diagnosed colon cancer and Lynch Syndrome. This recommendation is based on the increased risk of metachronous colorectal cancer in the syndrome. There is a paucity of studies regarding the benefits of more extended procedures compared to limited resections in the Lynch Syndrome. In this chapter, the surgical management of Lynch syndrome patients will be addressed including the rationale for limited and extended resections.


Lynch Syndrome HNPCC Surgery Segmental resection Abdominal colectomy Ileorectal anastomosis Prohpylactic Surgery 


  1. 1.
    Vasen HF, Möslein G, Alonso A, Bernstein I, Bertario L, Blanco I, et al. Guidelines for the clinical management of Lynch syndrome (hereditary non-polyposis cancer). J Med Genet. 2007;44(6):353–62.PubMedCrossRefGoogle Scholar
  2. 2.
    Fitzgibbons RJ Jr, Lynch HT, Stanislav GV, Watson PA, Lanspa SJ, Marcus JN, et al. Recognition and treatment of patients with hereditary nonpolyposis colon cancer (Lynch syndromes I and II). Ann Surg. 1987;206(3):289–95.PubMedCrossRefGoogle Scholar
  3. 3.
    Aarnio M, Mecklin JP, Aaltonen LA, Nyström-Lahti M, Järvinen HJ. Life-time risk of different cancers in hereditary non-polyposis colorectal cancer (HNPCC) syndrome. Int J Cancer. 1995;64(6):430–3.PubMedCrossRefGoogle Scholar
  4. 4.
    Box JC, Rodriguez-Bigas MA, Weber TK, Petrelli NJ. Clinical implications of multiple colorectal carcinomas in hereditary nonpolyposis colorectal carcinoma. Dis Colon Rectum. 1999;42(6):717–21.PubMedCrossRefGoogle Scholar
  5. 5.
    Natarajan N, Watson P, Silva-Lopez E, Lynch HT. Comparison of extended colectomy and limited resection in patients with Lynch syndrome. Dis Colon Rectum 2010;53(1):77–82.Google Scholar
  6. 6.
    de Vos tot Nederveen Cappel WH, Buskens E, van Duijvendijk P, Cats A, Menko FH, Griffioen G, et al. Decision analysis in the surgical treatment of colorectal cancer due to a mismatch repair gene defect. Gut. 2003;52(12):1752–5.PubMedCrossRefGoogle Scholar
  7. 7.
    Maeda T, Cannom RR, Beart RW Jr, Etzioni DA. Decision model of segmental compared with total abdominal colectomy for colon cancer in Hereditary Nonpolyposis Colorectal Cancer. J Clin Oncol 2010;28(7):1175–80.Google Scholar
  8. 8.
    Madden MV, Neale KF, Nicholls RJ, Landgrebe JC, Chapman PD, Bussey HJ, et al. Comparison of morbidity and function after colectomy with ileorectal anastomosis or restorative proctocolectomy for familial adenomatous polyposis. Br J Surg. 1991;78(7):789–92.PubMedCrossRefGoogle Scholar
  9. 9.
    de Vos tot Nederveen Cappel WH, Nagengast FM, Griffioen G, Menko FH, Taal BG, Kleibeuker JH, et al. Surveillance for hereditary nonpolyposis colorectal cancer: A long-term study on 114 families. Dis Colon Rectum. 2002;45(12):1588–94.PubMedCrossRefGoogle Scholar
  10. 10.
    Rodríguez-Bigas MA, Vasen HF, Pekka-Mecklin J, Myrhøj T, Rozen P, Bertario L, et al. Rectal cancer risk in hereditary nonpolyposis colorectal cancer after abdominal colectomy. International Collaborative Group on HNPCC. Ann Surg. 1997;225(2):202–7.PubMedCrossRefGoogle Scholar
  11. 11.
    Meagher A, Farouk R, Dozois RR, Kelly KA, Pemberton JH. J ileal pouch-anal anastomosis for chronic ulcerative colitis: complications and long-term outcome in 1310 patients. Br J Surg. 1998;85(6):800–3.PubMedCrossRefGoogle Scholar
  12. 12.
    Soravia C, Klein L, Berk T, O’Connor BI, Cohen Z, McLeod RS. Comparison of ileal pouch-anal anastomosis and ileorectal anastomosis in patients with familial adenomatous polyposis. Dis Colon Rectum. 1999;42(8):1028–33.PubMedCrossRefGoogle Scholar
  13. 13.
    Dozois E, Dozois RR. Familial Adenomatous Polyposis, in Mayo Clinic Gastrointestinal Surgery. In: Kelly KA, Sarr MG, Hinder RA, editors. Philadelphia: Saunders; 2004. p. 559–67.Google Scholar
  14. 14.
    Jass JR, Smyrk TC, Stewart SM, Lane MR, Lanspa SJ, Lynch HT. Pathology of hereditary non-polyposis colorectal cancer. Anticancer Res. 1994;14(4B):1631–4.PubMedGoogle Scholar
  15. 15.
    Lanspa SJ, Jenkins JX, Cavalieri RJ, Smyrk TC, Watson P, Lynch J, et al. Surveillance in Lynch syndrome: How aggressive? Am J Gastroenterol. 1994;89(11):1978–80.PubMedGoogle Scholar
  16. 16.
    Nugent KP, Spigelman AD, Phillips RK. Life expectancy after colectomy and ileorectal anastomosis for familial adenomatous polyposis. Dis Colon Rectum. 1993;36(11):1059–62.PubMedCrossRefGoogle Scholar
  17. 17.
    Syngal S, Weeks JC, Schrag D, Garber JE, Kuntz KM. Benefits of colonoscopic surveillance and prophylactic colectomy in patients with hereditary nonpolyposis colorectal cancer mutations. Ann Intern Med. 1998;129(10):787–96.PubMedCrossRefGoogle Scholar
  18. 18.
    Vasen HF, Wijnen JT, Menko FH, Kleibeuker JH, Taal BG, Griffioen G, et al. Cancer risk in families with hereditary nonpolyposis colorectal cancer diagnosed by mutation analysis. Gastroenterology. 1996;110(4):1020–7.PubMedCrossRefGoogle Scholar
  19. 19.
    Pocard M, Pomel C, Lasser P. Laparoscopic prophylactic surgery for HNPCC gene mutation carrier: has the time come? Lancet Oncol. 2003;4(10):637–8.PubMedCrossRefGoogle Scholar
  20. 20.
    Farrington SM, Lin-Goerke J, Ling J, Wang Y, Burczak JD, Robbins DJ, et al. Systematic analysis of hMSH2 and hMLH1 in young colon cancer patients and controls. Am J Hum Genet. 1998;63(3):749–59.PubMedCrossRefGoogle Scholar
  21. 21.
    Giardiello FM, Brensinger JD, Petersen GM. AGA technical review on hereditary colorectal cancer and genetic testing. Gastroenterology. 2001;121(1):198–213.PubMedCrossRefGoogle Scholar
  22. 22.
    Lamberti C, Kruse R, Ruelfs C, Caspari R, Wang Y, Jungck M, et al. Microsatellite instability-a useful diagnostic tool to select patients at high risk for hereditary non-polyposis colorectal cancer: A study in different groups of patients with colorectal cancer. Gut. 1999;44(6):839–43.PubMedCrossRefGoogle Scholar
  23. 23.
    Hendriks YM, Wagner A, Morreau H, Menko F, Stormorken A, Quehenberger F, et al. Cancer risk in hereditary nonpolyposis colorectal cancer due to MSH6 mutations: impact on counseling and surveillance. Gastroenterology. 2004;127(1):17–25.PubMedCrossRefGoogle Scholar
  24. 24.
    Schmeler KM, Lynch HT, Chen LM, Munsell MF, Soliman PT, Clark MB, et al. Prophylactic surgery to reduce the risk of gynecologic cancers in the Lynch syndrome. N Engl J Med. 2006;354(3):261–9.PubMedCrossRefGoogle Scholar
  25. 25.
    Guillem JG, Wood WC, Moley JF, Berchuck A, Karlan BY, Mutch DG, et al. ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes. J Clin Oncol. 2006;24(28):4642–60.PubMedCrossRefGoogle Scholar
  26. 26.
    Brown GJ, St John DJ, Macrae FA, Aittomäki K. Cancer risk in young women at risk of hereditary nonpolyposis colorectal cancer: Implications for gynecologic surveillance. Gynecol Oncol. 2001;80(3):346–9.PubMedCrossRefGoogle Scholar
  27. 27.
    Lindor NM, Petersen GM, Hadley DW, Kinney AY, Miesfeldt S, Lu KH, et al. Recommendations for the care of individuals with an inherited predisposition to Lynch syndrome: A systematic review. JAMA. 2006;296(12):1507–17.PubMedCrossRefGoogle Scholar
  28. 28.
    Dove-Edwin I, Boks D, Goff S, Kenter GG, Carpenter R, Vasen HF, et al. The outcome of endometrial carcinoma surveillance by ultrasound scan in women at risk of hereditary nonpolyposis colorectal carcinoma and familial colorectal carcinoma. Cancer. 2002;94(6):1708–12.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Anderson Cancer CenterThe University of TexasHoustonUSA

Personalised recommendations