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.
References in periodicals archive ?
Von Hippel-Lindau disease is a hereditary cancer syndrome caused by a germline mutation in or deletion of the VHL gene, and patients are at risk for developing tumors and fluid-filled sacs (cysts) in a number of organs.
Through the collection and evaluation of a family cancer history by a trained health care provider, patients and families at increased risk for a hereditary cancer syndrome can be identified, referred for genetic counseling and testing, and make informed decisions about options for cancer risk reduction (1).
Furthermore, there are situations in which the a priori likelihood of a hereditary cancer syndrome is increased, as in cases with multiple primaries known to be associated with a syndrome, such as endometrial cancer and colon cancer in the same patient, in individuals with rare tumors such as pheochromocytoma, or paraganglioma, or otherwise common malignancies such as basal cell carcinomas when present in strikingly elevated numbers, or those with uncharacteristically early ages of onset.
The first steps in determining whether a hereditary cancer syndrome is present in a family are to gather detailed family, medical, and lifestyle histories and to obtain details of cancer sites and histological features of the tumours.
In this statement "the evaluation for the presence of a hereditary cancer syndrome enables physicians to provide individualized and quantified assessment of cancer risk, as well as options for tailored screening and preventions strategies that may reduce morbidity associated with the development of malignancy" (Gynecol Oncol.
Claims for negligence arising from failure to diagnose the hereditary cancer syndrome, failure to offer the option of prophylactic mastectomy (which likely would have prevented the malignancy altogether), and ultimately delayed diagnosis of breast cancer were brought against all 3 ObGyns along with both genetic counselors and their supervising MFMs.
A negative BRCA test result simply means you don't face a higher-than-average risk for the disease due to a hereditary cancer syndrome.
Hereditary nonpolyposis colorectal cancer (HNPCC) is likely the most common hereditary cancer syndrome.
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.
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.

Full browser ?