The first step to determine your family cancer risk is to document your family history, by asking family members the type of cancer they had, and the age when the diagnosis was made. This information could prove to be valuable either now or in the years to come. Most people do not have a strong family history of cancer. Sometimes, people with a family history of cancer overestimate their own cancer risk. For these people, gathering family history information and discussing their concerns may help to decrease their cancer worry.

The Hereditary Cancer Program at the Siteman Cancer Center offers cancer risk assessment and education as well as genetic testing for individuals with a family history of cancer.

Understanding Hereditary Cancer

• Hereditary cancer is cancer that is inherited or passed on in a family.

• Unlike most cancer, hereditary cancer results from an abnormal alteration in a single gene.

• Approximately, 5-10 percent of all cancer is considered to be hereditary. For example, 5 percent of all women with breast cancer have a hereditary type of breast cancer.

• People with a family history of cancer may have a significantly increased risk to develop one or more types of cancer.

• Families may have hereditary cancer if there are multiple people in the family with cancer, including more than one generation of family members with cancer. In addition, people diagnosed at a young age are more likely to have hereditary cancer.

• Some types of hereditary cancer are more common among certain ethnic groups.

Specific Types of Hereditary Cancer
There are many different hereditary cancer syndromes.

The specific syndrome name does not indicate all of the cancer types for which family members may be at risk to develop. For example, families with a hereditary breast cancer syndrome also have an increased risk to develop ovarian cancer. Knowing the different cancers associated with a cancer syndrome allows family members to undergo earlier and more frequent cancer screening. See the glossary for a list of common hereditary cancer syndromes.

Cancer Risk Assessment and Education
Cancer risk assessment and education is a clinical evaluation for people with questions about their cancer risk based on their family history of cancer. A genetics doctor and a genetic counselor work with you to assess your cancer risk, to review your children''s or other family members'' cancer risk, to identify risk appropriate cancer screening or treatment options, and to coordinate genetic testing. You do not have to have genetic testing to pursue this assessment.

Who Should Consider Cancer Risk Assessment?

• Any person with a family history of cancer who is interested in learning more about his or her family- based cancer risk.

• Any person who has a family member with hereditary cancer.

• Any person who is considering prophylactic medical or surgical therapy because of his or her family history of cancer.

• Any person who is interested in learning more about genetic testing for cancer.

What Happens During the Cancer Risk Assessment Appointment?
Prior to the appointment, a genetic counselor helps you to document your family history and to collect available medical records on family members with cancer. This information is needed to assess your family cancer risk. Appointments may be arranged if medical records cannot be obtained.

During the appointment, you will meet with a genetics physician and a genetic counselor to discuss your cancer risk, recommend appropriate cancer screening practices, review available medical or surgical treatments, and coordinate genetic testing.

After the appointment, a detailed summary of your evaluation will be provided to you and your doctor.

What is Genetic Testing?
Genetic testing is used to first identify the cancer gene alteration responsible for the increased cancer risk in a family. Family members who have a cancer gene alteration have an increased chance to develop cancer and may benefit from earlier or more frequent cancer screening. In some cases, medical or surgical treatments are available before cancer develops.

Genetic testing is coordinated by the genetics doctor and genetic counselor and is performed using a blood sample. Not all people who pursue cancer risk assessment decide to have genetic testing.

Glossary
List of some hereditary cancer syndromes

Diagnosis of a hereditary cancer syndrome begins with a thorough evaluation of the family history. For each syndrome listed below, there is a 50 percent chance to pass on the altered gene to each child. Each child who inherits the gene has an increased chance to develop cancer.

Breast-ovarian cancer syndrome 1 results from an abnormal change in the BRCA1 gene. Family members have an estimated 80-90 percent lifetime chance to develop breast cancer and a 40-60 percent lifetime chance to develop ovarian cancer. Mammography screening and ovarian cancer screening is recommended for all female family members.

Breast-ovarian cancer syndrome 2 results from an abnormal change in the BRCA2 gene. Family members have an estimated 80-90 percent lifetime chance to develop breast cancer and an estimated 20-30 percent lifetime chance for ovarian cancer. Other cancers are associated with this syndrome including prostate cancer and melanoma. Mammography screening and ovarian cancer screening is recommended for all female family members.

Familial adenomatous polyposis is a hereditary colon cancer syndrome that results from an abnormal alteration in the APC gene. Individuals develop hundreds to thousands of polyps. Since any of the hundreds of polyps may develop into cancer, removal of the colon is recommended. Polyps may develop in other areas of the digestive system, requiring lifelong cancer screening.

Familial melanoma results from an abnormal alteration in at least one of three genes. Families with familial melanoma have an increased chance to develop melanoma as well as pancreatic and brain tumors. Monthly skin self-exams and clinical examinations by a dermatologist every six months is recommended.

Hereditary nonpolyposis colon cancer (HNPCC) is a hereditary colon cancer syndrome resulting from an abnormal change in one of at least six genes. Families with HNPCC have a 70-80 percent lifetime chance to develop colon cancer. Female family members have a 40-60 percent lifetime chance to develop uterine cancer. Colon and uterine cancer screening is recommended to begin at a young age.

Multiple endocrine neoplasia 2A (MEN2A) results from an abnormal alteration in the RET gene. Family members have an increased chance to develop medullary thyroid cancer, hyperparathyroidism (overactive parathyroid gland) and tumors of the adrenal gland (known as pheochromocytoma). Removal of the thyroid gland is recommended.

Von Hippel Lindau syndrome results from an abnormal change in the VHL gene. Families with VHL have an increased risk to develop kidney cancer, tumors of the adrenal gland (known as pheochromocytoma) as well as brain and spinal tumors. Tumors found on the retina (located in the back of the eye) are characteristic of this syndrome. Evaluations of the eye, kidneys, brain and spinal cord are recommended on a yearly basis.

Act Now
Talk with your family members about your family history and then share this information with your doctor. Knowledge of your family''s medical history may be important in your ongoing medical care.

For more information or to schedule an appointment with a Siteman Cancer Center specialist at Barnes-Jewish Hospital and Washington University School of Medicine, call 314-TOP-DOCS (314-867-3627) or toll-free at 866-867-3627.