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Surgical Management of Hereditary Colorectal Cancer Syndromes

  • Johannes Dörner
  • Mahmoud Taghavi Fallahpour
  • Gabriela Möslein
Chapter

Abstract

Due to the advances in molecular genetic diagnostics of adenomatous polyposis variants as well as nonpolyposis syndromes, identification of patients with a genetic predisposition and their at-risk relatives is becoming increasingly important in clinical practice. Knowledge of the specific risk profile is gaining significance and requires a clinically differentiated approach to correctly identify the indications for prophylactic and extended therapeutic surgery. In this chapter, decision-making and the technical details of the operation for different colorectal cancer (CRC) syndromes are outlined. Besides the more commonly known polyposis syndromes, such as familial adenomatous polyposis (FAP), surgeons should be able to clinically distinguish between attenuated and classical variants of FAP and be aware of MUTYH-associated polyposis (MAP) and the newly described polyposis polymerase proofreading-associated polyposis (PPAP), among others. Surgeons should be familiar with the specific indications and extent of surgery for prophylactic organ removal in the lower gastrointestinal tract to knowledgeably advise patients and enable them to make informed decisions. Moreover, reconstructive options after proctocolectomy and novel innovative techniques in proctectomy, such as the transanal total mesorectal excision (TaTME), are discussed.

Keywords

Timing of surgery Ileal pouch-anal anastomosis Ileorectal anastomosis Quality of life Desmoid disease Prophylactic surgery 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Johannes Dörner
    • 1
  • Mahmoud Taghavi Fallahpour
    • 2
  • Gabriela Möslein
    • 3
  1. 1.Klinik für Allgemein- und Viszeralchirurgie, HELIOS University Hospital Witten/HerdeckeWuppertalGermany
  2. 2.Private University Witten/HerdeckeWittenGermany
  3. 3.Center for Hereditary Tumours, HELIOS University Hospital Witten/HerdeckeWuppertalGermany

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