The management of rectal cancer is complex enough in itself but even more complicated in the setting of a hereditary cancer syndrome. Although the cancer treatment as such is similar to the one in sporadic cancer and based on cancer stage, tumor location, the patient's overall presentation and condition, the presence of an underlying disease raises additional concerns about functional aspects and the risk of metachronous lesions. We reviewed the role of multimodality treatment and the timing and extent of the planned surgeries. Rectal cancer in the setting of hereditary cancer syndromes (eg, hereditary nonpolyposis colorectal cancer, familial adenomatous polyposis) is not genuinely handled with a different approach, except when an ileal pouch anal anastomosis is planned as the neo-adjuvant modality should always be chosen to avoid the detrimental effect of radiation on an ileal pouch.
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