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.
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