Management of Hereditary Colorectal Cancer: A Multidisciplinary Approach

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