title: Targeted therapy for hereditary cancer syndromes: hereditary breast and ovarian cancer syndrome, Lynch syndrome, familial adenomatous polyposis, and Li-Fraumeni syndrome.
abstract: Cancer genetics has rapidly evolved in the last two decades. Understanding and exploring the several genetic pathways in the cancer cell is the foundation of targeted therapy. Several genomic aberrations have been identified and their role in carcinogenesis is being explored. In contrast to most cancers where these mutations are acquired, patients with hereditary cancer syndromes have inherited genomic aberrations. The understanding of the molecular pathobiology in hereditary cancer syndromes has advanced dramatically. In addition, many molecularly targeted therapies have been developed that could have potential roles in the treatment of patients with hereditary cancer syndromes. In this review, we outline the presentation, molecular biology, and possible targeted therapies for two of the most widely recognized hereditary cancer syndromes -- hereditary breast and ovarian cancer syndrome and hereditary non-polyposis colorectal cancer syndrome (Lynch syndrome). We will also discuss other syndromes such as familial adenomatous polyposis and Li-Fraumeni syndrome (TP53).
authors.affiliation: Department of Medicine Division of Hematology/Oncology, University of Cincinnati, Cincinnati, OH 45267, USA.
authors.forename: Rishi
authors.initials: R
authors.lastname: Agarwal
authors.affiliation: UT Health, University of Texas - Houston, Houston, TX 77030, USA.
authors.forename: Sarah
authors.initials: S
authors.lastname: Liebe
authors.affiliation: CollabRx Inc., San Francisco, CA 94104, USA.
authors.forename: Michelle L
authors.initials: ML
authors.lastname: Turski
authors.affiliation: CollabRx Inc., San Francisco, CA 94104, USA.
authors.forename: Smruti J
authors.initials: SJ
authors.lastname: Vidwans
authors.affiliation: Department of Investigational Cancer Therapeutics, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
authors.forename: Filip
authors.initials: F
authors.lastname: Janku
authors.affiliation: Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84112, USA.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Garrido-Laguna
authors.affiliation: Banner MD Anderson Cancer Center, Gilbert, AZ 85234, USA.
authors.forename: Javier
authors.initials: J
authors.lastname: Munoz
authors.affiliation: Center for Personalized Cancer Therapy, Moores Cancer Center, University of California, San Diego, CA 92093, USA.
authors.forename: Richard
authors.initials: R
authors.lastname: Schwab
authors.affiliation: Vall d'Hebron Institut d'Oncologia and Universitat Autonoma of Barcelona, Barcelona, 08035, Spain.
authors.forename: Jordi
authors.initials: J
authors.lastname: Rodon
authors.affiliation: Center for Personalized Cancer Therapy, Moores Cancer Center, University of California, San Diego, CA 92093, USA.
authors.forename: Razelle
authors.initials: R
authors.lastname: Kurzrock
authors.affiliation: Department of Investigational Cancer Therapeutics, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
authors.forename: Vivek
authors.initials: V
authors.lastname: Subbiah
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000188:drug therapy* / Q000235:genetics; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000188:drug therapy* / Q000235:genetics; D061325:Hereditary Breast and Ovarian Cancer Syndrome / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D016864:Li-Fraumeni Syndrome / Q000188:drug therapy* / Q000235:genetics; D009154:Mutation
chemical_list: D000970:Antineoplastic Agents