Any location in the body beyond the abdomen (e.g., neck, chest, leg, arm, thigh, etc.).
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“Familial adenomatous polyposis (F.A.P.) is a rare inherited cancer predisposition syndrome characterized by hundreds to thousands of precancerous colorectal polyps (adenomatous polyps). If left untreated, affected individuals inevitably develop cancer of the colon and/or rectum at a relatively young age. FAP is inherited in an autosomal dominant manner and caused by abnormalities (mutations) in the APC[anchor link to APC entry in Glossary] gene. Mutations in the APC gene cause a group of polyposis conditions that have overlapping features: familial adenomatous polyposis, Gardner syndrome, Turcot syndrome and attenuated FAP.” (Source: National Organization for Rare Disorders (NORD))
“A fibroblast is a type of cell that contributes to the formation of connective tissue, a fibrous cellular material that supports and connects other tissues or organs in the body. Fibroblasts secrete collagen proteins that help maintain the structural framework of tissues. They also play an important role in healing wounds.” (Source: NIH National Human Genome Research Institute)
“The first treatment given for a disease. It is often part of a standard set of treatments, such as surgery followed by chemotherapy and radiation. When used by itself, first-line therapy is the one accepted as the best treatment. If it doesn’t cure the disease or it causes severe side effects, other treatments may be added or used instead. Also called induction therapy, primary therapy, and primary treatment.” (Source: National Cancer Institute)
Gamma-secretase inhibitors (GSIs) are targeted treatments that selectively inhibit a protein called gamma secretase and reduce activation of a pathway that is believed to play a role in the growth of desmoid tumors. These medical therapies are pills that can be taken orally at home. GSIs were originally developed as Alzheimer’s therapies, but have been repurposed as anticancer agents given their inhibition of specific biological processes that are altered in cancer.
Research has shown that gamma-secretase inhibitors have shown benefit in desmoid tumors by shrinking or slowing down the growth and improving patient’s symptoms. As with all therapies, gamma secretase inhibitors can also cause side effects that can vary from person to person.
