[Surgical Management of Hereditary Colorectal Cancer Syndromes]

Colorectal cancer (CRC) is among the most common malignancies worldwide. Approximately 10% of all CRCs are caused by monogenic hereditary tumour syndromes, collectively referred to as hereditary colorectal cancers (hCRC). The increasing use of molecular diagnostics - such as microsatellite instability (MSI) testing - is expected to significantly raise the detection rate in the coming years. hCRC can be broadly divided into polyposis syndromes (e.g. FAP, MAP) and non-polyposis syndromes (primarily Lynch syndrome). Surgical management must be tailored to the specific syndrome, and must balance oncological safety with long-term functional outcomes. In FAP, timely prophylactic colectomy is essential, whereas in MAP, the surgical strategy depends on polyp burden and tumour location. For rare polyposis syndromes such as NTHL1-, POLE-, or POLD1-associated syndromes, evidence-based recommendations are lacking, and treatment should follow FAP/aFAP protocols. Lynch syndrome is associated with a significantly increased risk of metachronous tumours. In this case, surgical strategies must be re-evaluated that consider emerging immuno-oncologic therapies, such as checkpoint inhibition in MSI-positive tumours. Surgical care for patients with hCRC should be provided at specialised centres, including genetic counselling, and be guided by interdisciplinary tumour board discussions.