title: The evolution of colorectal cancer genetics-Part 2: clinical implications and applications.
abstract: The genetic understanding of colorectal cancer (CRC) continues to grow, and it is now estimated that 10% of the population has a known hereditary CRC syndrome. This article will examine the evolving surgical and medical management of hereditary CRC syndromes, and the impact of tumor genetics on therapy. This review will focus on the most common hereditary CRC-prone diseases seen in clinical practice, which include Lynch syndrome (LS), familial adenomatous polyposis (FAP) & attenuated FAP (AFAP), MutYH-associated polyposis (MAP), and serrated polyposis syndrome (SPS). Each section will review the current recommendations in the evaluation and treatment of these syndromes, as well as review surgical management and operative planning. A highly detailed multigeneration cancer family history with verified genealogy and pathology documentation whenever possible, coupled with germline mutation testing when indicated, is critically important to management decisions. Although caring for patients with these syndromes remains complex, the application of this knowledge facilitates better treatment of both individuals and their affected family members for generations to come.
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Andrew T
authors.initials: AT
authors.lastname: Schlussel
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Ronald A
authors.initials: RA
authors.lastname: Gagliano
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Susan
authors.initials: S
authors.lastname: Seto-Donlon
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Faye
authors.initials: F
authors.lastname: Eggerding
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Timothy
authors.initials: T
authors.lastname: Donlon
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Berenberg
authors.affiliation: 1 Department of Surgery, Tripler Army Medical Center, Honolulu, HI, USA ; 2 University of Arizona Cancer Center at Dignity Health-St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA ; 3 Genetics Laboratory, Huntington Medical Research Institutes, Pasadena, CA, USA ; 4 Ohana Genetics, Inc., Honolulu, HI, USA ; 5 Department of Cell & Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA ; 6 Department of Oncology, Tripler Army Medical Center, Honolulu, HI, USA ; 7 Hereditary Cancer Center, Creighton University School of Medicine, Omaha, NE, USA.
authors.forename: Henry T
authors.initials: HT
authors.lastname: Lynch
keywords: Surgical oncologic management; hereditary colorectal cancer (hereditary CRC); medical oncologic management