Journal: Zentralblatt fur Chirurgie
This publication reviews hereditary colorectal cancers (hCRC), which account for about 10% of colorectal cancer cases and arise from monogenic syndromes.
The syndromes are categorized into:
- Polyposis syndromes (e.g., FAP, MAP)
- Non-polyposis syndromes, mainly Lynch syndrome
Molecular diagnostics, such as microsatellite instability testing, are improving detection rates.
Surgical management must be customized to each syndrome, balancing oncologic control with functional outcomes:
- For FAP, prophylactic colectomy is crucial.
- MAP treatment depends on polyp burden and tumor location.
- Rare polyposis syndromes are managed similarly to FAP due to limited data.
- Lynch syndrome patients require tailored surgery because of a high risk of metachronous tumors.
Emerging immunotherapies, such as checkpoint inhibitors, are considered for MSI-positive cancers.
Optimal care involves specialized centers offering genetic counseling and multidisciplinary team planning.