Abstract
While it is true that only a small proportion of patients develop gastrointestinal cancer in the setting of an inherited syndrome, it is important for the medical oncologist to recognize this unique group of patients for several reasons. First, genetic testing of the tumor can have implications for the patient’s prognosis and may help tailor chemotherapy in this age of personalized cancer medicine. Second, affected patients are often at risk for other malignancies either within the gastrointestinal tract or involving other organ systems and, as such, are in need of close clinical surveillance. Lastly, the oncologist is in an excellent position to encourage genetic counseling and testing of at-risk family members. In this chapter we will review familial adenomatous polyposis (FAP) syndrome and hereditary nonpolyposis colon cancer (HNPCC), aka Lynch syndrome, in some detail. We will also discuss polyposis syndromes other than FAP, namely, Peutz-Jegher syndrome and juvenile polyposis as well as hereditary diffuse gastric cancer and inherited pancreatic cancer syndromes.
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Thirumurthi, S., Vilar, E., Lynch, P.J. (2019). Hereditary Gastrointestinal Cancers. In: Yalcin, S., Philip, P. (eds) Textbook of Gastrointestinal Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-18890-0_33
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DOI: https://doi.org/10.1007/978-3-030-18890-0_33
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