Abstract
Patients with Lynch syndrome are predisposed to developing colorectal cancer and a
variety of extracolonic malignancies, at a young age. The management of rectal cancer
in the setting of Lynch syndrome is a complex clinical scenario that requires the
expertise of a multidisciplinary management team. In this review, we delve into the
approach for rectal cancer in these patients, and specifically focus on several key
aspects of treatment. Some unique aspects of rectal cancer in Lynch syndrome include
the decision between proctectomy alone versus total proctocolectomy with or without
an ileal pouch, the utility of chemotherapy and immunotherapy, nonoperative rectal
cancer management, and the management of rectal polyps. Throughout, we highlight the
delicate interplay between future cancer risk reduction and quality of life optimization.
Keywords
Lynch syndrome - mismatch repair - rectal cancer - colorectal cancer - hereditary
rectal cancer
