Surgical management of hereditary colorectal cancer

Abstract

Colorectal cancer (CRC) is one of the most common solid malignancies worldwide. Although sporadic CRC represents the most common form, genetic alterations is increasingly being identified in a relevant portion of patients with CRC. Familial CRC describes an increased incidence of adenomatous polyps and CRC in first - degree relatives. Hereditary CRC is defined by the identification of deleterious mutations in known predisposing genes. Typical hereditary syndromes with predisposition to CRC include: hereditary non-polyposis colon cancer or Lynch syndrome, familial adenomatous polyposis, attenuated familial adenomatous polyposis, Peutz-Jeghers syndrome and MUTYH associated polyposis. Newly identified genetic alterations with increased risk for CRC include: PPAP, NAD, MSH3 and NTHL1. The diagnosis, surveillance and optimal surgical management of patients with hereditary predisposition to CRC warrant a good understanding of the genetic syndrome in question. Prophylactic surgery must be segregated from symptom-related procedures depending on the syndrome in question. The need for extended surgical procedures must be made in an individualized manner based on gene and gender. The patient should play an active role in the surgical decision-making. Minimally invasive access should be the preferred approach and postoperative quality of life must be seen as a primary outcome measure.

Keywords

Hereditary colorectal cancer / hereditary non-polyposis colon cancer / Lynch Syndrome / familial adenomatous polyposis / MUTYH associated polyposis / polyposis / proctocolectomy / virtual ileostomy

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