Gastrointestinal Cancer Syndromes

Lynch Syndrome, also called Hereditary Nonpolyposis Colorectal Cancer, or HNPCC, is associated with

  • Colorectal polyps
  • Colorectal cancer
  • Endometrial (uterine) cancer
  • Other cancers such as gastric, biliary tract, urinary tract, ovarian, small bowel and pancreatic cancers are sometimes seen
  • Turcot syndrome is a variant of Lynch syndrome that also involves brain cancer
  • Muir-Torre syndrome is a variant of Lynch syndrome that also involves sebaceous tumors

 

Familial Adenomatous Polyposis (FAP) is a hereditary cancer syndrome that involves

  • A predisposition to develop colorectal polyps and colorectal cancer if the polyps are not removed
  • An increased risk to develop hundreds or thousands of colon polyps
  • Stomach, douodenal and pancreatic cancer is sometimes seen

There is also a weakened form of FAP, called attenuated familial adenomatous polyposis that involves fewer polyps and a lower risk for colorectal cancer. This syndrome also involves increased risks for stomach cancer and thyroid cancer, among other cancers.

MYH-Associated Polyposis is an autosomal recessive inherited genetic condition, which means that both parents passed on a change in the MYH gene that is associated with

  • Colon, stomach or duodenal polyps
  • Colorectal cancer
  • Non-cancerous tumors of the jaw or sebaceous glands

Peutz-Jeghers Syndrome (PJS) is a rare hereditary cancer syndrome associated with

  • Cancers of the stomach, small intestines, colon, pancreas, breast, lung, uterus and ovary
  • A specific type of colon polyps called hamartomatous polyps
  • Freckling on specific parts of the body, such as the lips and nose. This freckling often disappears during puberty

Juvenile Polyposis Syndrome (JPS) is a rare hereditary cancer syndrome that involves the development of a specific type of polyp called a juvenile polyp throughout the gastrointestinal tract. The term "juvenile polyp" refers to the type of polyp, not the age at which the polyp occurs. People with JPS have increased risks of stomach cancer and colorectal cancer.

 

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