hereditary cancer syndrome

hereditary cancer syndrome

Any of a group of often AD conditions characterized by tumors that are often site-specific, of early onset and multiple and/or bilateral; HCSs include nevoid basal cell carcinoma syndrome, dysplastic nevus–B-K mole syndrome, Cowden syndrome, familial polyposis, Gardner syndrome, Gorlin syndrome, Li-Fraumeni syndrome, MEN I, MEN II. See Beckwith Wiedemann syndrome, Cancer family, Dysplastic nevus sydrome, Familial malignancy, Hereditary neoplasmia, Hereditary preneoplasia, Li-Fraumeni syndrome.
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ELST is strongly associated with VHL disease, a hereditary cancer syndrome.
The critical first step in the identification of a hereditary cancer syndrome is to obtain a careful family history of cancer.
This is an example of a phenocopy, a person who develops a tumor in an organ at risk in hereditary cancer syndrome and does not have hereditary cancer syndrome.
In addition, a well-established link exists between the hereditary cancer syndrome von Hippel-Lindau (VHL) disease and HIF-2 leading to increased susceptibility to clear-cell renal carcinoma (CC-RCC).
Afterward, an assessment that involved hypothetical scenarios about hereditary cancer syndrome revealed increased genetic knowledge and more appropriate referral recommendations.
In most instances, the pedigree of a family affected by a hereditary cancer syndrome reveals cancer risk to be inherited as an autosomal-dominant disorder, in which approximately half the offspring of each mutation carrier develops a specific cancer associated with the syndrome.
Incomplete family histories and smaller families may be less sensitive for assessing risk of a hereditary cancer syndrome.
The American Society of Clinical Oncologists has identified MEN 2 as a well-defined hereditary cancer syndrome for which genetic testing is considered standard management.
Ten of the 16 women in her study had family histories that suggested they could be at risk for a hereditary cancer syndrome, yet they reported they had not received a cancer risk assessment, said Dr.
Patients at increased risk for hereditary cancer syndromes should be referred to a cancer genetics specialist for further assessment and counseling, according to the opinion from ACOG's Committee on Genetics (Obstet.
Objective: Defining and characterising the defective genes in hereditary cancer syndromes has advanced our understanding of cellular function and disease mechanisms.
Genetic mutations associated with more than 50 hereditary cancer syndromes - including those discovered at the University of Utah for melanoma, colon and breast cancer - have been identified.

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