Management of Hereditary Colorectal Cancer. A Multidisciplinary Approach

Description

Contents Contributors Introduction 1: Early-Age-of-Onset Colorectal Carcinoma: An Emerging Public Health Issue Increasing Incidence of CRC in Young Adults Is a Global Health Issue Reasons for Increasing Incidence of CRC in Young Adults Are Not Known Currently Known Risk Factors for CRC May Not Be Relevant for Young Adults Current Data on Risk Factors for CRC in Young Adults Are Limited Population-Based CRC Screening Programs Do Not Cover Young Adults Current CRC Screening Guidelines Are Limited in Use for Young Adults Most CRC in Young Adults Present in Late Stages Burden of Young Adult CRC Is Huge Risk-Based Screening Is a Way to Reduce the CRC Burden in Young Adults References 2: Management of Hamartomatous Polyps Introduction Peutz-Jeghers Syndrome Radiologic Imaging in PJS Endoscopy in PJS Surveillance Management of PJS Juvenile Polyposis Syndrome Surveillance and Management PTEN Hamartoma Tumor Syndrome Summary References 3: Familial Adenomatous Polyposis: Prophylactic Management of the Colon and Rectum Introduction Clinical Presentation Diagnosis and Screening Genetics Chemoprevention Surgical Management IRA Versus IPAA Mucosectomy and Handsewn IPAA Versus Stapled IPAA Minimally Invasive (MIS) Versus Open Surgery Type of Ileal Pouch Postoperative Surveillance Quality of Life Conclusion References 4: FAP Surveillance Post IPAA or IRA Introduction Total Proctocolectomy and Ileal Pouch-Anal Anastomosis Endoscopic Screening Surveillance Recommendations Following Ileal Pouch-Anal Anastomosis Medical Management of Adenomatous Polyps Following Ileal Pouch-Anal Anastomosis Surgical Management of Polyps Following Ileal Pouch-Anal Anastomosis Cancer Management Following Ileal Pouch-Anal Anastomosis Total Abdominal Colectomy with Ileorectal Anastomosis Surveillance and Management of the Rectum Following Ileorectal Anastomosis Desmoid Tumors and Completion Proctectomy Following Ileorectal Anastomosis Conclusions References 5: Familial Adenomatous Polyposis: Management of Upper Gastrointestinal Polyps Introduction Gastric Neoplasia Gastric Fundic Gland Polyps Gastric Adenomas Duodenal Neoplasia Duodenal Adenomas Ampullary Adenomas Management of Gastric and Duodenal Neoplasms Screening and Surveillance Gastric Polyps Duodenal Adenomas Ampullary Adenomas Endoscopic Resection of Gastric and Duodenal Neoplasms Endoscopic Resection of Ampullary Adenomas Surgical Resection of Duodenal and Ampullary Neoplasms Chemoprevention Sulindac Celecoxib Agents Under Investigation Summary References 6: Desmoids in Familial Adenomatous Polyposis What Are Desmoids? Epidemiology Desmoids in FAP Desmoid Formation Growth and Progression Risk Factors for Desmoid Formation Genetic Gender Trauma Interrelationship Between Desmoids and Surgery Diagnosis Apparently Sporadic Desmoid Desmoid in Patients with FAP Imaging Treatment Drug Treatment Cytotoxic Chemotherapy Other Treatments Surgery Other Therapies Treatment Strategy Abdominal Wall and Extra-Abdominal Desmoid Intra-abdominal Desmoid Multiple Sites Acute Presentation of Intra-abdominal Desmoid Challenges References 7: Hepatoblastoma in Infants Born to Parents with Familial Adenomatous Polyposis Introduction Incidence of FAP in Hepatoblastoma Patients and of Hepatoblastoma in FAP Patients Genetic Overlap Between Hepatoblastoma and Familial Adenomatous Polyposis Presentation, Prognosis, and Treatment of Hepatoblastoma Benefits of Screening for FAP in Hepatoblastoma Patients and for Hepatoblastoma in FAP Patients Conclusion References 8: MYH-Associated Polyposis: Manifestations, Management, and Surveillance of the Colorectum Clinical Presentation Genetic Counseling Endoscopic Screening and Surveillance Surgical Management of the Colon and Rectum Summary References 9: Lynch Syndrome: Management of the Colon, What Operation? Introduction Risk CRC Risk Prophylactic Operations Colon and Rectum Other Organs Treatment of Endoscopically Unmanageable Polyps Management of Primary CRC Choices of Operation for Primary CRC: Segmental Colectomy Versus Extended Colectomy (Total or Subtotal Colectomy) Impact of MMR Gene Mutation on Extent of CRC Resection Impact of MMR Gene Mutation on Other Organ Resection Impact of Age on Extent of CRC Resection Impact of Age on Other Organ Resection Management of Secondary CRC Summary References 10: Lynch Syndrome: Management of Rectum, What Operation? Introduction Framework for Treatment Planning Key Elements in the Clinical Assessment of LS Patients with Rectal Cancer Clinical Stage IV Rectal Cancer Choice of Operation Clinical Stage II and III Disease Tri-Modality Therapy Choice of Operation Curative-Intent Proctectomy Laparoscopic and Laparoscopic Transanal TME Robotic TME Multimodality Therapy Extended Resection Concomitant Prophylactic Surgery Clinical Stage I and Early Stage II Disease Choice of Operation Local Excision Conclusion References Index