Von Hippel-Lindau (VHL) disease is a familial cancer syndrome
characterized by benign or malignant tumors which may involve more than one system.
Rhabdomyosarcoma in children: epidemiologic study and identification of a familial cancer syndrome
Management of extraocular sebaceous carcinoma is complicated by its association with the familial cancer syndrome
, Muir-Torre Syndrome.
An estimated 15% to 20% of colorectal cancers are related to a familial cancer syndrome
Lhermitte-Duclos disease is closely associated with Cowden's syndrome, a rare, autosomal-dominant, familial cancer syndrome
characterized by multiple hamartomas and neoplasms of ectodermal, mesodermal, and endodermal origin.
However, there are still many families whose history suggests an inherited risk for cancer, but for whom the specific genes associated with the familial cancer syndrome
have not yet been identified.
For example, if a patient's grandmother had breast cancer when she was 65, an aunt had cervical cancer at age 25, and an uncle had lung cancer because he smoked, that may indeed be a strong family history of cancer, but when you ask if this is consistent with a familial cancer syndrome
, it is not," Dr.
We're not suggesting that every familial cancer syndrome
involves p53," he says.
In the past decade the genetic contributions to cancer syndromes have become cleared  and about 500 familial cancer syndromes
have been described (a comprehensive list can be found at http://www.
Three established, yet disparate, models are used to translate predictive cancer genomics into primary care medical practice for patients at risk for hereditary or familial cancer syndromes
Young-onset cancer is a hallmark of many familial cancer syndromes
, but it was not clear whether family members of young-onset familial pancreatic cancer patients were at greater risk than family members of older-onset patients.
These may be genes associated with other familial cancer syndromes
or may largely be associated with increased breast cancer risk.