FAP, gastric cancer, and genetic counseling featuring children and young adults: a family study and review

• Published in: Familial cancer 2010-12, Vol.9 (4), p.581-588
• Main Authors: Lynch, Henry T., Snyder, Carrie, Davies, Janine M., Lanspa, Stephen, Lynch, Jane, Gatalica, Zoran, Graeve, Victoria, Foster, Jason
• Format: Article
• Language: English
• Subjects: Adenomatous Polyposis Coli - diagnosis; Adenomatous Polyposis Coli - genetics; Adolescent; Adult; Aged; Biomedical and Life Sciences; Biomedicine; Cancer Research; Child; Child, Preschool; Epidemiology; Female; Gastric Fundus; Genetic Counseling; Human Genetics; Humans; Infant; Infant, Newborn; Male; Middle Aged; Pedigree; Polyps - diagnosis; Polyps - genetics; Stomach Neoplasms - diagnosis; Stomach Neoplasms - genetics; Young Adult
• ISSN: 1389-9600; 1573-7292
• DOI: 10.1007/s10689-010-9352-7

Familial adenomatous polyposis is a highly complex and multifaceted colorectal cancer prone disorder which is often significantly confounded by extracolonic cancers inclusive of gastric cancer, a significant problem in the Orient. Gastric cancer in familial adenomatous polyposis is heavily influenced by fundic gland polyps which are often so voluminous as to defy effective endoscopic surveillance. This study involves more than two decades of investigation of an attenuated familial adenomatous polyposis family where gastric cancer posed an early diagnostic problem because it was obscured by multiple fundic gland polyps. Fundic gland polyps are common in familial adenomatous polyposis and attenuated familial adenomatous polyposis and, if voluminous, may interfere with effective endoscopic gastric cancer surveillance. This family is believed to be the first of its type reported with focus upon education and genetic counseling in the setting of a family information service. Cancer control in familial adenomatous polyposis may be partially resolved through use of familial colorectal cancer registries, with greater attention to family history and its interpretation, genetic counseling, and clinical translation for diagnosis and management.