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Prophylaktische Chirurgie beim hereditären nichtpolypösen kolorektalen Karzinom

Prophylactic surgery for hereditary nonpolyposis colorectal cancer

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Zusammenfassung

Das häufigste kolorektale Prädispositionssyndrom ist das Lynch-Syndrom, auch HNPCC („hereditary nonpolyposis colorectal cancer“) genannt. Die Option einer prophylaktischen Chirurgie hängt von der Penetranz des genetischen Defektes und der Heterogenität der Krankheitsausprägung ab. Da bei HNPCC 20% der Mutationsträger nie ein Karzinom ausbilden, ist eine rein prophylaktische Dickdarmresektion nicht indiziert. Bei Auftreten eines kolorektalen Karzinoms hingegen ergibt sich die Frage nach einem Benefit durch Erweiterung der onkologischen Resektion im Sinne einer totalen Kolektomie bzw. einer (restaurativen) Proktokolektomie. Diese Patienten sollten in eine von der Krebshilfe geförderten prospektiv-randomisierten Studie eingebracht werden (http://www.hnpcc-studie.de). Auch für das Endometrium und den Magen wird bei hoher Karzinominzidenz und unsicherer Vorsorge die Option einer prophylaktischen Intervention diskutiert.

Abstract

The most frequent hereditary colorectal cancer predisposition is Lynch syndrome, or hereditary nonpolyposis colorectal cancer. The option of prophylactic surgery relies on the penetrance of the genetic defect and the heterogeneity of the condition. Since 20% of all mutation carriers never move on to develop cancer, the purely prophylactic setting is not indicated. However, when colorectal cancer is diagnosed, the question arises if the patient may benefit from extended surgery — total colectomy or (restorative) proctocolectomy. These patients should be entered into the ongoing prospective-randomized study by German Cancer Aid (http://www.hnpcc-studie.de). Due to substantially increased cancer risk and poor surveillance options, the endometrium and stomach are also subject to the question of prophylactic intervention.

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Literatur

  1. Aarnio M, Mecklin JP, Aaltonen LA, Nystrom-Lahti M, Jarvinen HJ (1995) Life-time risk of different cancers in hereditary non-polyposis colorectal cancer (HNPCC) syndrome. Int J Cancer 64:430–433 [EBM III]

    PubMed  Google Scholar 

  2. Aarnio M, Sankila R, Pukkala E et al. (1999) Cancer risk in mutation carriers of DNA-mismatch-repair genes. Int J Cancer 81:214–218

    Article  PubMed  Google Scholar 

  3. Bekanntmachungen der Bundesärztekammer (2003): Richtlinien zur prädiktiven genetischen Diagnostik. Dtsch Ärztebl 100: A1297–1305

    Google Scholar 

  4. Boks DE, Trujillo AP, Voogd AC, Morreau H, Kenter GG, Vasen HF (2002) Survival analysis of endometrial carcinoma. Int J Cancer 102:198–200

    Article  PubMed  Google Scholar 

  5. Burke W, Daly M, Garber J, Botkin J et al. (1997) Recommendations for follow-up care of individuals with inherited predisposition of cancer. JAMA 277:915–919

    Article  PubMed  Google Scholar 

  6. Camilleri-Brennan J, Steele RJ (1998) Quality of life after treatment for rectal cancer. B J Surg 85:1036–1043

    Article  Google Scholar 

  7. Duijvendijk P, Slors JFM, Taat CW. Oosterveld P, Spangers AG, Obertop H., Vasen HF (2000) Quality of life after total colectomy with ileorectal anastomosis or proctocolectomy and ileal pouch-anal anastomosis for familial adenomatous polyposis. Br J Surg 87:590–596

    Article  PubMed  Google Scholar 

  8. Dunlop MG (2002) Guidance on gastrointestinal surveillance for hereditary non-polyposis colorectal cancer, familial adenomatous polypolis, juvenile polyposis, and Peutz-Jeghers syndrome. Gut 51 [Suppl 5]: V21–27

  9. Dunlop MG, Farrington SM, Carothers AD et al. (1997) Cancer risk associated with germline DNA mismatch repair gene mutations. Hum Mol Gen 6:105–110

    Article  PubMed  Google Scholar 

  10. Hatcher MB, Fallowfield LJ, A‚Hern R (2001) The psychosocial impact of bilateral prophylactic mastectomy: prospective study. BMJ 322:76

    Article  PubMed  Google Scholar 

  11. Hendriks Y, Wagner A, Morreau H et al. (2004) Cancer Risk in hereditary nonpolyposis colorectal cancer due to MSH6 mutations: impact on counselling and surveillance. Gastroenterology:127:17–25

    Google Scholar 

  12. Jarvinen HJ, Aarnio M, Mustonen H et al. (2000) Controlled 15-year trial on screening for colorectal cancer in families with hereditary nonpolyposis colorectal cancer. Gastroenterology 118:829–834

    Article  PubMed  Google Scholar 

  13. Koller M, Kussman J, Lorenz W et al. (1996) Symptom reporting in cancer patient. The role of negative affect an experienced social stigma. Cancer 77:983–995

    Article  PubMed  Google Scholar 

  14. Lerman C, Daly M, Masny A, Balshelm A. (1994) Attitudes about genetic testing for breast-ovarian cancer susceptibility. J Clin Oncol 12:843–850

    PubMed  Google Scholar 

  15. Lu KH, Broaddus RR (2001) Gynecological tumors in hereditary nonpolyposis colorectal cancer: We know they are common--now what? Gynecol Oncol 82:221–222

    Article  PubMed  Google Scholar 

  16. Lynch HT, de la Chapelle A (2003) Hereditary colorectal cancer. N Engl J Med 348:919–932

    Article  PubMed  Google Scholar 

  17. Lynch HT, Smyrk T, Lanspa SJ, Marcus JN, Kriegler M, Lynch JF, Appelman HD (1988) Flat adenomas in a colon cancer-prone kindred. J Natl Cancer Inst 80:278–282

    PubMed  Google Scholar 

  18. Lynch H, Grady W, Suriano G, Huntsmann D (2005) Gastric cancer: New Genetic developments: J Surg Oncol 90:114–133

    Google Scholar 

  19. Mecklin JP, Järvinen H (1993) Treatment and follow-up strategies in hereditary nonpolyposis colorectal carcinoma. Dis Colon Rectum 36:927–929

    Article  PubMed  Google Scholar 

  20. Olschwang S, Laurent-Puig P, Eisinger F, Millat B (2005) An alternative to prophylactic colectomy for colon cancer precention in HNPCC syndrome. Gut 54:169–172

    Article  Google Scholar 

  21. Papadopoulos N, Lindblom A (1999) Molecular basis of HNPCC: mutations of MMR genes. Hum Mut 10:89–99

    Article  Google Scholar 

  22. Renkonen-Sinisalo L, Sipponen P, Aarnio M et al. (2002) No support for endoscopic surveillance for gastric cancer in hereditary non-polyposis colorectal cancer. Scand J Gastroenterol 37:574–577

    Article  PubMed  Google Scholar 

  23. Rodriguez-Bigas MA, Vasen HF, Lynch HT et al. (1998) Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma. International Collaborative Group on HNPCC. Cancer 83:240–244

    Article  PubMed  Google Scholar 

  24. Schmiegel W, Pox C, Adler G, Fleig W et al. (2004) S3-Guidelines Conference „Colorectal Carcinoma“ 2004. Z Gastroenterol 42:1129–1177

    Article  PubMed  Google Scholar 

  25. Schulmann et al. (2004)

  26. Syngal S, Weeks JC, Schrag D (1998) Benefits of colonoscopic surveillance and prophylactic colectomy in patients with hereditary nonpolyposis colorectal cancer mutations. Ann Intern Med 129:787–796

    PubMed  Google Scholar 

  27. Umar A, Boland CR, Terdiman JP, Syngal S et al. (2004) Revised Bethesda Guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability. J Natl Cancer Inst 96:261–268

    PubMed  Google Scholar 

  28. Vasen HF, Offerhaus GJ, den Hartog Jager FC et al. (1990) The tumour spectrum in hereditary non- polyposis colorectal cancer: a study of 24 kindreds in the Netherlands. Int J Cancer 46:31–34 [EBM III]

    PubMed  Google Scholar 

  29. Vasen HF, Watson P, Mecklin JP et al. (1999) New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative group on HNPCC. Gastroenterology 116:1453–1456

    Article  PubMed  Google Scholar 

  30. Vasen HFA, de Vos tot Nederveen Cappel WH (2005) An alternative to prophylactic colectomy for colon cancer prevention in HNPCC syndrome. Gut 54:1501–1502

    Article  PubMed  Google Scholar 

  31. de Vos tot Nederveen Cappel WH, Nagengast FM, Griffioen G, Menko FH, Taal BG, Kleibeuker JH, Vasen HF (2002) Surveillance for hereditary nonpolyposis colorectal cancer: a long-term study on 114 families. Dis Colon Rectum 45:1588–1594

    Article  PubMed  Google Scholar 

  32. de Vos tot Nederveen Cappel WH, Buskens E, Duijvendijk van P et al. (2003) Decision analysis in the surgical treatment of colorectal cancer due to a mismatch repair gene defect. Gut 52:1752–1755

    Article  PubMed  Google Scholar 

  33. Zhang YZ, Sheng JQ, Li SR; Zhang H (2005) Clinical phenotype and prevalence of hereditary nonpolyposis colorectal cancer syndrome in Chinese population. World J Gastroenterol 11:1481–1488

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Allgemein- und Viszeralchirurgie, Coloproktologie, St. Josefs Hospital Bochum-Linden

    G. Möslein

  2. Koordinierungszentrum für Klinische Studien (KKS), Universitätsklinikum Düsseldorf

    C. Ohmann & M. Wenzel

  3. Allgemein- und Viszeralchirurgie, Coloproktologie, St. Josefs Hospital Bochum-Linden, Axstraße 35, 44879 , Bochum

    G. Möslein

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  1. G. Möslein
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Correspondence to G. Möslein.

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Möslein, G., Ohmann, C. & Wenzel, M. Prophylaktische Chirurgie beim hereditären nichtpolypösen kolorektalen Karzinom. Chirurg 76, 1135–1144 (2005). https://doi.org/10.1007/s00104-005-1114-y

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