Skip to main content
SpringerLink
Log in

Surgical Approach for Colorectal Cancer in Patients with Lynch Syndrome

  • Chapter
  • First Online:
Lynch Syndrome

  • 372 Accesses

Abstract

Lynch syndrome (LS) is caused by pathogenic variants of one of DNA mismatch repair genes (MLH1, MSH2, MSH6, PMS2) or 3′ deletion of EPCAM in the germline. Colorectal cancer (CRC) is a hallmark of the syndrome, and the cumulative risk of CRC ranges from 10 to 46% by 70 years old, depending on the gene affected. Moreover, despite the high cumulative risk of subsequent CRC (metachronous CRC) after the first CRC is managed with surgery, the optimal surgical treatment is controversial. Prophylactic colorectal resection prior to CRC development is not recommended regardless of the type of surgery. Recent studies and our analysis show that the risk of metachronous CRC is considerably higher following segmental colectomy as compared to extended colectomy among patients with genetically proven LS. However, overall survival did not differ between patients with two different surgical procedures. The choice of surgical procedures for the first rectal cancer is complicated because of insufficient data available and thus needs further investigations. Surgical procedures for first CRC in patients with LS may depend on various factors that may vary among individuals, including the timing of genetic diagnosis (before or after the first CRC development), age at CRC diagnosis, site of CRC, genes affected, and expected quality of life. Individuals with genetically proven LS must be counselled prior to any surgical intervention for first CRC. In summary, an extended colectomy may be currently the most effective surgical technique to reduce the risk of metachronous colon cancer.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 16.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 129.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Edwards BK, Ward E, Kohler BA, Eheman C, Zauber AG, Anderson RN, Jemal A, Schymura MJ, Lansdorp-Vogelaar I, Seeff LC, van Ballegooijen M, Goede SL, Ries LA. Annual report to the nation on the status of cancer, 1975–2006, featuring colorectal cancer trends and impact of interventions (risk factors, screening, and treatment) to reduce future rates. Cancer. 2010;116:544–73.

    Article  Google Scholar 

  2. Lynch HT, Lynch PM, Lanspa SJ, Snyder CL, Lynch JF, Boland CR. Review of the Lynch syndrome: history, molecular genetics, screening, differential diagnosis, and medicolegal ramifications. Clin Genet. 2009;76:1–18.

    Article  CAS  Google Scholar 

  3. Møller P, Seppälä T, Bernstein I, Holinski-Feder E, Sala P, Evans DG, Lindblom A, Macrae F, Blanco I, Sijmons R, Jeffries J, Vasen H, Burn J, Nakken S, Hovig E, Rødland EA, Tharmaratnam K, de Vos Tot Nederveen Cappel WH, Hill J, Wijnen J, Green K, Lalloo F, Sunde L, Mints M, Bertario L, Pineda M, Navarro M, Morak M, Renkonen-Sinisalo L, Frayling IM, Plazzer JP, Pylvanainen K, Sampson JR, Capella G, Mecklin JP, Möslein G, Mallorca Group (http://mallorca-group.eu). Cancer incidence and survival in Lynch syndrome patients receiving colonoscopic and gynaecological surveillance: first report from the prospective Lynch syndrome database. Gut. 2017;66:464–72.

    Article  Google Scholar 

  4. Vasen HF, Blanco I, Aktan-Collan K, Gopie JP, Alonso A, Aretz S, Bernstein I, Bertario L, Burn J, Capella G, Colas C, Engel C, Frayling IM, Genuardi M, Heinimann K, Hes FJ, Hodgson SV, Karagiannis JA, Lalloo F, Lindblom A, Mecklin JP, Møller P, Myrhoj T, Nagengast FM, Parc Y, Ponz de Leon M, Renkonen-Sinisalo L, Sampson JR, Stormorken A, Sijmons RH, Tejpar S, Thomas HJ, Rahner N, Wijnen JT, Järvinen HJ, Möslein G, Mallorca group. Revised guidelines for the clinical management of Lynch syndrome (HNPCC): recommendations by a group of European experts. Gut. 2013;62:812–23.

    Article  CAS  Google Scholar 

  5. Kim TJ, Kim ER, Hong SN, Kim YH, Huh JW, Park YA, Cho YB, Yun SH, Kim HC, Lee WY, Kim K, Kim K, Chang DK. Survival outcome and risk of metachronous colorectal cancer after surgery in Lynch syndrome. Ann Surg Oncol. 2017;24:1085–92.

    Article  Google Scholar 

  6. Hiatt MJ, Casey MJ, Lynch HT, Snyder CL, Stacey M, Walters RW. Efficacy of proximal colectomy for surgical management of right-sided first colorectal cancer in Lynch Syndrome mutation carriers. Am J Surg. 2018;216:99–105.

    Article  Google Scholar 

  7. Win AK, Parry S, Parry B, Kalady MF, Macrae FA, Ahnen DJ, Young GP, Lipton L, Winship I, Boussioutas A, Young JP, Buchanan DD, Arnold J, Le Marchand L, Newcomb PA, Haile RW, Lindor NM, Gallinger S, Hopper JL, Jenkins MA. Risk of metachronous colon cancer following surgery for rectal cancer in mismatch repair gene mutation carriers. Ann Surg Oncol. 2013;20:1829–36.

    Article  Google Scholar 

  8. Vasen HF, Watson P, Mecklin JP, Lynch HT. New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative group on HNPCC. Gastroenterology. 1999;116:1453–6.

    Article  CAS  Google Scholar 

  9. Heneghan HM, Martin ST, Winter DC. Segmental vs extended colectomy in the management of hereditary nonpolyposis colorectal cancer: a systematic review and meta-analysis. Color Dis. 2015;17:382–9.

    Article  CAS  Google Scholar 

  10. Anele CC, Adegbola SO, Askari A, Rajendran A, Clark SK, Latchford A, Faiz OD. Risk of metachronous colorectal cancer following colectomy in Lynch syndrome: a systematic review and meta-analysis. Color Dis. 2017;19:528–36.

    Article  CAS  Google Scholar 

  11. Malik SS, Lythgoe MP, McPhail M, Monahan KJ. Metachronous colorectal cancer following segmental or extended colectomy in Lynch syndrome: a systematic review and meta-analysis. Fam Cancer. 2018;17:557–64.

    Google Scholar 

  12. Lindor NM. Familial colorectal cancer type X: the other half of hereditary nonpolyposis colon cancer syndrome. Surg Oncol Clin N Am. 2009;18:637–45.

    Article  Google Scholar 

  13. 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;53:77–82.

    Article  Google Scholar 

  14. Parry S, Win AK, Parry B, Macrae FA, Gurrin LC, Church JM, Baron JA, Giles GG, Leggett BA, Winship I, Lipton L, Young GP, Young JP, Lodge CJ, Southey MC, Newcomb PA, Le Marchand L, Haile RW, Lindor NM, Gallinger S, Hopper JL, Jenkins MA. Metachronous colorectal cancer risk for mismatch repair gene mutation carriers: the advantage of more extensive colon surgery. Gut. 2011;60:950–7.

    Article  Google Scholar 

  15. Renkonen-Sinisalo L, Seppälä TT, Järvinen HJ, Mecklin JP. Subtotal colectomy for colon cancer reduces the need for subsequent surgery in Lynch syndrome. Dis Colon Rectum. 2017;60:792–9.

    Article  Google Scholar 

  16. Stupart DA, Goldberg PA, Baigrie RJ, Algar U, Ramesar R. Surgery for colonic cancer in HNPCC: total vs segmental colectomy. Color Dis. 2011;13:1395–9.

    Article  CAS  Google Scholar 

  17. Pylvänäinen K, Lehtinen T, Kellokumpu I, Järvinen H, Mecklin JP. Causes of death of mutation carriers in Finnish Lynch syndrome families. Familial Cancer. 2012;11:467–71.

    Article  Google Scholar 

  18. de Vos tot Nederveen Cappel WH, Buskens E, van Duijvendijk P, Cats A, Menko FH, Griffioen G, Slors JF, Nagengast FM, Kleibeuker JH, Vasen HF. Decision analysis in the surgical treatment of colorectal cancer due to a mismatch repair gene defect. Gut. 2003;52:1752–5.

    Article  Google Scholar 

  19. 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;129:787–96.

    Article  CAS  Google Scholar 

  20. Kalady MF. Surgical management of hereditary nonpolyposis colorectal cancer. Adv Surg. 2011;45:265–74.

    Article  Google Scholar 

  21. Kalady MF, Lipman J, McGannon E, Church JM. Risk of colonic neoplasia after proctectomy for rectal cancer in hereditary nonpolyposis colorectal cancer. Ann Surg. 2012;255:1121–5.

    Article  Google Scholar 

  22. You YN, Chua HK, Nelson H, Hassan I, Barnes SA, Harrington J. Segmental vs. extended colectomy: measurable differences in morbidity, function, and quality of life. Dis Colon Rectum. 2008;51:1036–43.

    Article  Google Scholar 

  23. Haanstra JF, de Vos Tot Nederveen Cappel WH, Gopie JP, Vecht J, Vanhoutvin SA, Cats A, van der Zaag-Loonen HJ, Langers AM, Bergmann JH, van de Meeberg PC, Dekker E, Kleibeuker JH, Vasen HF, Nagengast FM, van Duijvendijk P. Quality of life after surgery for colon cancer in patients with Lynch syndrome: partial versus subtotal colectomy. Dis Colon Rectum. 2012;55:653–9.

    Article  Google Scholar 

  24. Lindor NM, Petersen GM, Hadley DW, Kinney AY, Miesfeldt S, Lu KH, Lynch P, Burke W, Press N. Recommendations for the care of individuals with an inherited predisposition to Lynch syndrome: a systematic review. JAMA. 2006;296:1507–17.

    Article  CAS  Google Scholar 

  25. Kalady MF, McGannon E, Vogel JD, Manilich E, Fazio VW, Church JM. Risk of colorectal adenoma and carcinoma after colectomy for colorectal cancer in patients meeting Amsterdam criteria. Ann Surg. 2010;252:507–11.

    PubMed  Google Scholar 

  26. Herzig DO, Buie WD, Weiser MR, You YN, Rafferty JF, Feingold D, Steele SR. Clinical practice guidelines for the surgical treatment of patients with Lynch syndrome. Dis Colon Rectum. 2017;60:137–43.

    Article  Google Scholar 

  27. Burn J, Gerdes AM, Macrae F, Mecklin JP, Moeslein G, Olschwang S, Eccles D, Evans DG, Maher ER, Bertario L, Bisgaard ML, Dunlop MG, Ho JW, Hodgson SV, Lindblom A, Lubinski J, Morrison PJ, Murday V, Ramesar R, Side L, Scott RJ, Thomas HJ, Vasen HF, Barker G, Crawford G, Elliott F, Movahedi M, Pylvanainen K, Wijnen JT, Fodde R, Lynch HT, Mathers JC, Bishop DT, CAPP2 Investigators. Long-term effect of aspirin on cancer risk in carriers of hereditary colorectal cancer: an analysis from the CAPP2 randomised controlled trial. Lancet. 2011;378:2081–7.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan

    Hideyuki Ishida & Keiichiro Ishibashi

  2. Department of Gastroenterological Surgery, Faculty of Medicine, Kagawa University, Kita-gun, Kagawa, Japan

    Kensuke Kumamoto

Authors
  1. Hideyuki Ishida
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Keiichiro Ishibashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kensuke Kumamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hideyuki Ishida .

Editor information

Editors and Affiliations

  1. Specially Appointed Adviser, Cancer Treatment Center, Toyonaka Municipal Hospital, Osaka, Japan

    Naohiro Tomita

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Ishida, H., Ishibashi, K., Kumamoto, K. (2020). Surgical Approach for Colorectal Cancer in Patients with Lynch Syndrome. In: Tomita, N. (eds) Lynch Syndrome. Springer, Singapore. https://doi.org/10.1007/978-981-15-6891-6_10

Download citation

  • DOI: https://doi.org/10.1007/978-981-15-6891-6_10

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-15-6890-9

  • Online ISBN: 978-981-15-6891-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation