Gilda Radner Familial Ovarian Cancer Registry
OVARIAN CANCER AWARENESS!
Ovarian Cancer Awareness
Cancer is a disease in which cells in the body grow out of control. Cancer is always named for the part of the body where it starts, even if it spreads to other body parts later. When cancer starts in the ovaries, it is called ovarian cancer. Women have two ovaries that are located in the pelvis, one on each side of the uterus. The ovaries make female hormones and produce eggs.
Ovarian cancer will be diagnosed in about one out of 55 women (approximately 1.8%). Ovarian cancer counts for about 3% of all cancers in women.
Ovarian cancer symptoms are often not taken seriously because they are similar to other women’s bodily complaints. The signs of ovarian cancer include:
• A feeling of being bloated
• Vague abdominal and pelvic discomfort
• Gastrointestinal symptoms such as gas, nausea, indigestion
• Constipation, diarrhea, frequent urination
• Back pain and fatigue
• Discomfort during sex
• Vaginal bleeding
Although many women have these symptoms of ovarian cancer, they are often overlooked. If any of these ovarian cancer signs are experienced almost every day and persist for weeks, they could be an early warning of ovarian cancer and should be brought to your doctor’s attention. Gilda Radner had every one of these symptoms for over a year prior to her diagnosis. Early detection can make the difference. Over 70% of all women with ovarian cancer will not be diagnosed until the disease has spread beyond the ovary. This is because the symptoms of early ovarian cancer are often vague and can mimic other common medical problems.
Unfortunately, there is no simple reliable ovarian cancer test available to screen women. Women need to stay in touch with their bodies and report changes to their doctors, because early detection is crucial. In a recent study more than 81% of women diagnosed with ovarian cancer had one of these symptoms before diagnosis. The Gilda Radner Familial Ovarian Cancer Registry has been recommending for over two decades that women experiencing continued symptoms of ovarian cancer have a combination of ovarian cancer tests that include
• Pelvic examination
• Vaginal ultrasound
• CA125 test
A Pap smear does not detect ovarian cancer. It is a screening tool to detect cervical cancer.
Ovarian cancer is most common in women who have already gone through menopause. The average age for developing ovarian cancer is 61 years of age. In families with two or more diagnoses of ovarian cancer, the cancer can be diagnosed at earlier ages, most commonly in their 40s.
Ovarian cancer prognosis is best when the disease is found early. Over 70% of all women with ovarian cancer will not be diagnosed until the disease has spread beyond the ovary. This is because the symptoms of early ovarian cancer can be vague and mimic other common medical problems. For the small number of women who are fortunate enough to have their cancer diagnosed before it has spread beyond the ovary, the chance for cure is 85 to 90%. However, for the majority of women in whom the disease has spread beyond the ovary, the chance of living for five years after the diagnosis is between 20 and 25%.
Specific risk factors or ovarian cancer causes are not known, but risk factors that may increase your chances of getting ovarian cancer may include:
• High fat diet
• Never having children
• Infertility, or not having children until late in life
• Using infertility drugs but not becoming pregnant
• Starting your periods at a young age, or going through menopause at an older than average age
• Use of talcum powder on the genital area
• Caucasian race
• Have an Eastern European (Ashkenazi) Jewish background.
• Family history of ovarian cancer, breast cancer, or colon cancer
• Peronal history of breast, uterine or colon cancer
Of these ovarian cancer risk factors, the most significant is a family history of ovarian cancer and /or breast cancer (on either your mother’s side of the family or your father’s side of the family). Having one close relative with ovarian cancer increases a woman’s risk of developing ovarian cancer by nearly three times. Having additional family members with breast cancer, ovarian cancer or colon cancer increases the risk even further.
Most women with ovarian cancer risk factors will never actually get ovarian cancer. Even with significant factors such as family history, the overall chances of getting ovarian cancer are still small.
Fortunately, there are a number of factors that are associated with lowering the risk of ovarian cancer.
• Use of birth control pills
• Having multiple children
• Breast feeding
• Tubal ligation
• Having the ovaries removed (prophylactic oophorectomy)
If you have only one close relative, such as a mother or sister, with a history of ovarian cancer, the overwhelming chances are that you will not develop ovarian cancer. However, you are at increased risk, and special testing by your doctor may be indicated.
Once a year to every six months the Gilda Radner Familial Ovarian Cancer Registry recommends women who have at least one close relative with ovarian cancer have a
• Pelvic examination
• Vaginal ultrasound
• CA125 test
There are almost 40 different types of ovarian cancer. However, nine out of 10 ovarian cancer patients have epithelial tumors, which begin in the tissue of the surface of the ovary (epithelium). These are called adenocarcinomas – a malignant (cancerous) tumor of epithelial origin which begins in glandular tissue, Serous adenocarcinoma is seen most often, followed by endometrioid, mucinous and clear cell adenocarcinomas. Carcinomas of borderline malignancy are a subgroup of serous and mucinous adenocarcinomas, which are usually less aggressive and have a significantly higher cure rate than serous and mucinous adenocarcinomas.
The only definitive way to diagnose ovarian cancer is surgery to remove the tumor for laboratory evaluation. Fortunately, there are tests to help determine if surgery is needed. In addition to a pelvic exam, pelvic and vaginal ultrasound of the ovaries can often (but not always) help distinguish between malignant and benign (noncancerous) tumors. Cystic tumors (i.e., no solid areas suggesting cancer) are usually benign. When solid areas are seen on ultrasound, the chances of cancer increase. CA125 levels (a tumor marker in the blood), which are elevated in eight out of 10 women with advanced (stage III and stage IV) disease and in one out of two women with cancer localized in the ovary (stage I), can be determined by a simple blood test. However, CA125 levels can also be elevated in benign conditions – endometriosis, pelvic inflammatory disease of the tubes and ovaries, uterine fibroids, pregnancy – and sometimes in cancer of the pancreas and the gastrointestinal tract.
Stage refers to how far the disease has advanced. Accurate staging is important in treatment planning because the prognosis (outcome) worsens as the stage increases. Generally, there are four stages of ovarian cancer.
Stage I: The Cancer is limited to the ovary or ovaries
* Stage IA: The tumor is limited to inside of one ovary
* Stage IB: The tumor is limited to the inside of both ovaries
* Stage IC: The tumor is limited to one or both ovaries. In addtion, it appears on the surface of the ovary, a fluid-filled capsule
has burst or cancer cells are found in the abdominal fluid.
Stage II: The cancer is one or both ovaries and has spread to other parts of the pelvis.
* Stage IIA: The tumor has spread to the uterus, fallopian tubes or both
* Stage IIB: The cancer has spread to the bladder, rectum or colon
* Stage IIC: The cancer tumor has spread to any of the above. Also, it appears on the surface of the ovary, a fluid-filled capsule
has burst or cancer cells are found in the abdominal fluid.
Stage III: The cancer is in one or both ovaries and has spread to nearby lymph nodes or other abdominal organs, not including the liver
* Stage IIIA: The tumor has spread to the lining of the abdomen but cannot be seen. The cancer has not spread to the lymph
nodes
* Stage IIIB: The cancer has sprread to the abdomen and is visible (less than two centimeters, about 3/4 of an inch in size).
The cancer has not spread to the lymph nodes.
* Stage IIIC: The cancer has spread into the abdomen and the deposits measure largers than two centimeters. The cancer has
spread to the lymph nodes.
Stage IV: The cancer has spread to the lung, liver or other distant organs.
Recurrent Ovarian Cancer: The cancer has come back after it has been treated. It may appear in other parts of the body, but is still considered ovarian cancer.
Surgery is needed for all stages of ovarian cancer, and when the surgeon (gynecologic oncologist) can document that cancer is limited to the ovary, it may be the only treatment needed. To document this, four areas within the abdominal cavity are evaluated: 1) the undersurface of the diaphragm; 2) the omentum (a fatty apron that hangs down from the colon); 3) lymph nodes along the abdominal aorta; and 4) pelvic lymph nodes. The abdominal cavity is also washed with a saline solution and the cells are stained to identify floating cancer cells not visible to the naked eye. For stages II, III, and IV, maximal tumor removal, ideally when surgery is performed by a gynecologic oncologist, results in the best survival rate.
Most women will get chemotherapy. The most important chemotherapy (drug treatment) agents for ovarian cancer are Platinum compounds and Taxanes. These medications are usually given intravenously (through a vein) every three to four weeks, for six treatments. Patients are evaluated at each treatment and have a pelvic examination, C125 test and blood work. If the CA125 level was elevated before, and is falling during chemotherapy, the treatment is almost certainly effective. If the CA125 level rises significantly during chemotherapy, it usually means that the treatment is not effective. Some women receive intraperitoneal chemotherapy (through a small catheter inserted into their abdominal [peritoneal] cavity). Intraperitoneal chemotherapy is often used when only very small deposits of cancer remain within the abdominal cavity after primary surgery.
There are other promising chemotherapy drugs available. Topotecan, Gemcitabine, Hexamethylmelamine, Tamoxifen, Doxil, or oral etoposide are effective in some women. In some patients, newer agents such as Avastin (an anti-angiogenic agent), are used to cut off blood supply to the tumor.
The BRCA1 and BRCA2 genes are responsible for many cases of familial ovarian cancer and familial breast cancer. Genes are small pieces of DNA, the material that acts as a master “blueprint” for all the cells in your body. Your genes determine such things as what color hair and eyes you have, how tall you are, and what you look like on the inside. They also instruct the body how to build all the chemical substances in your body that keep you running smoothly. Sometimes there is an error in one of your genes that causes it not to do its job properly. This can lead to disease and is called a “genetic defect”.
The BRCA1 and BRCA2 genes make a chemical substance that helps your body prevent cancer. Most women have two normal copies of the BRCA1 gene, or BRCA2 gene both of which produce this cancer preventing substance. Some women have a genetic defect in one of their two BRCA1 genes or BRCA2 genes and don’t produce a normal amount of this cancer fighting substance. These women are at very high risk of getting breast or ovarian cancer, as high as 85-90% over the course of a lifetime.
You inherit one copy of each of your genes from your mother and a second copy of each of your genes from your father. (This is why you look about half like your mother, and half like your father). If one of your parents has a defective BRCA1 gene or BRCA2 gene there is a 50% chance you may inherit their defective copy, and 50% chance you may inherit their normal copy. If you inherit a defective BRCA1 gene, or BRCA2 gene, then each of your children has 50% chance of inheriting it from you.
Although there is a test to detect a defective BRCA1 or BRCA2 gene, such a test is not recommended for all women. Women with a strong family history of ovarian cancer should consult their physicians about the test. It is best that genetic testing start with the family member diagnosed with cancer.
All women with a genetic mutation for BRCA1 or BRCA2 should consider removal of their ovaries after childbearing. For other women at high risk for ovarian cancer, because of a strong family history of ovarian cancer who choose not to undergo genetic testing or who do not carry a mutation for BRCA1 or BRCA2 should consider the surgery in a case by case basis. The removal of the ovaries (oophorectomy) can be accomplished as a simply outpatient surgery using a device called a laparoscope. There is only minimal discomfort, and in most cases a woman can return to work in two to three days. Because there is still a small risk of a similar type of cancer of the lining of the abdominal cavity, called primary peritoneal cancer, women who have their ovaries removed should continue to be seen by a doctor every six months and should continue routinely have the CA125 blood test. Women who have had their ovaries removed may want to continue on hormone replacement therapy (HRT), but need to take a cautious approach, in consultation with her own physician, due to the recent findings from the Women’s Health Initiative.
Women who have two or more close relatives diagnosed with ovarian cancer are asked to join the Gilda Radner Familial Ovarian Cancer Registry. By doing so, they can be assured of being kept informed of the very latest developments in familial ovarian cancer research. JOIN THE REGISTRY TODAY.
ABOUT THE REGISTRY
The Familial Ovarian Cancer Registry was established in 1981 by M. Steven Piver, M.D., former chairman of the Department of Gynecologic Oncology at Roswell Park Cancer Institute. In May 1990, the Registry was renamed to honor the memory of comedian, Gilda Radner, who died of ovarian cancer in 1989. Ms. Radner’s husband, film actor Gene Wilder, is honorary chairman of the Registry.
The Registry is a national computer tracking system that stores data for women with two or more close relatives who have been diagnosed with ovarian cancer and offers education, information and a Helpline with peer support for women at high risk (family history) of ovarian cancer.
The Gilda Radner Familial Ovarian Registry is pursuing research into causes of familial cancer in collaboration with investigators at Roswell Park Cancer Institute, Stanford University School of Medicine and Cambridge University. Our goals are to identify new genes associated with familial ovarian cancer, thereby improving genetic and psychosocial counseling for individuals and families and to characterize lifestyle choices (i.e., oral contraceptive use, hormone replacement therapy, number of pregnancies) that reduce ovarian cancer risk in women who may be more susceptible to the disease. We hope to acquire information that will lead to better methods for detecting ovarian cancer, for reliable predictive testing for cancer predisposition and ultimately, preventing the disease in future generations.
To date, the Registry has amassed the data for over 4,500 women who have been diagnosed with ovarian cancer in more than 1,850 families with two or more members with ovarian cancer.
Roswell Park Cancer Institute was founded in 1898 and is one of the oldest comprehensive cancer centers in the world. It is dedicated to providing total care for cancer patients, conducting research into the causes, treatment and prevention of cancer and to public and professional education.
Gilda Radner Familial Ovarian Cancer Registry
Roswell Park Cancer Institute
Elm and Carlton Streets
Buffalo NY 14263
1-800-OVARIAN
716-845-4503
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Ovarian Cancer Surgery
The surgery needed for ovarian cancer is called laparotomy. It involves making an incision into the abdominal cavity. The doctor is able to see the tumor, ovary and abdominal area. During the surgery the doctor will remove as much of the tumor as possible or may take a biopsy (smaller sample of the tissue).
Gynecologic Oncologist
A Gynecologic oncologist is an obstetrician/gynecologist who specializes in the diagnosis and treatment of women with cancer of the reproductive organs. Specifically, the gynecologic oncologist treats cancer of the ovary, endometrium, uterus, cervix, vagina, vulva and trophoblastic disease. In order to become a gynecologic oncologist in the United States, a physician must first complete an approved, 4-year residency program in obstetrics and gynecology. Following this, he/she must complete a 2–4 year clinical fellowship in gynecologic oncology. The additional training during fellowship provides the skills needed for optimal care of women with a gynecologic cancer.
How does the CA 125 blood test work to detect ovarian cancer?
Cancer antigen 125 is a substance that is produced in the fallopian tubes, uterus, cervix, and the lining the lining of the chest and abdominal cavities (the pleura and the peritoneum). The CA 125 test measures a sugar protein that may be released when cells are inflamed or damaged. CA 125 levels are usually measured by a blood test, but can also be detected in fluid from the abdominal and chest cavities. Levels under 35 kU/ml are considered normal.
Ovarian cancer cells may produce an excess of these protein molecules, as may some other cancers, including cancer of the fallopian tube or endometrial cancer (cancer of the lining of the uterus). Occasionally an elevated CA 125 test indicates other benign activity not associated with cancer, such as menstruation, pregnancy, or endometriosis. However, in early stage ovarian cancer, this molecule may not necessarily be released. Thus, the test is not an effective screening test.
The CA 125 test may be used diagnostically in combination with other tests such as vaginal ultrasound, and can also be used to monitor the progress of patients with cancer: increasing levels may indicate a recurrence, while decreasing levels may indicate a response to treatment.
Vaginal/transvaginal ultrasound or sonogram
An ultrasound uses sound waves to show what is inside your body. It has several uses, including finding tumors in the pelvis, and is painless. The transvaginal ultrasound (sonogram) can be used to aid in diagnosing ovarian cancer or cancer of the uterus. Since it can show an ovarian abnormality that doesn't exist, it is often used with the CA-125 blood test to diagnose ovarian cancer.
Pelvic exam
A pelvic exam is an exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. The doctor or nurse inserts one or two lubricated, gloved fingers of one hand into the vagina and the other hand is placed over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. A speculum is also inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease.
Ovarian cancer is usually advanced when detected by a pelvic exam.
Women’s Health Initiative
The WHI was launched in 1991 and consisted of a set of clinical trials and an observational study, which together involved 161,808 generally healthy postmenopausal women.
The clinical trials were designed to test the effects of postmenopausal hormone therapy, diet modification, and calcium and vitamin D supplements on heart disease, fractures, and breast and colorectal cancer.
The hormone trial had two studies: the estrogen-plus-progestin study of women with a uterus and the estrogen-alone study of women without a uterus. (Women with a uterus were given progestin in combination with estrogen, a practice known to prevent endometrial cancer.) In both hormone therapy studies, women were randomly assigned to either the hormone medication being studied or to placebo. Those studies have now ended. The women in these studies are now participating in a follow-up phase, which will last until 2010.
The FDA recommends that hormone therapy be used at the lowest doses for the shortest duration needed to achieve treatment goals. Postmenopausal women who use or are considering using hormone therapy should discuss the possible benefits and risks to them with their physicians.
Hormone Replacement Therapy (HRT)
During menopause, your ovaries decrease production of the female hormones estrogen and progesterone. This decline in hormones puts a permanent end to menstruation and fertility, but it can also cause hot flashes, mood swings, vaginal dryness and urinary problems. The solution? For decades, doctors routinely eased these symptoms with hormone replacement therapy — medications containing female hormones to replace the ones the body is no longer making. And it was widely believed that boosting estrogen levels after menopause could also ward off heart disease and osteoporosis, while improving quality of life and keeping women young.
Then, in 2002, a large clinical trial called the Women's Health Initiative (WHI) reported that hormone therapy actually posed more health risks than benefits for women in the clinical trial. As the number of health hazards attributed to hormone therapy grew, doctors became less likely to prescribe it. And up to two-thirds of women on the therapy discontinued its use, often without talking to their doctors.
Today, there's plenty of confusion about hormone replacement therapy, which is now commonly called hormone therapy. The truth is that hormone therapy is not the magical cure for aging that it was once believed to be, but it's still the most effective treatment for unpleasant menopausal symptoms for most women. If you're facing menopause, learn more about the benefits and the risks of hormone therapy.
Prophylactic Oophorectomy
In an oophorectomy, a surgeon removes both your ovaries, the almond-shaped organs on each side of your uterus. Your ovaries contain eggs and secrete the hormones that control your reproductive cycle. Removing your ovaries greatly reduces the amount of the hormones estrogen and progesterone circulating in your body. This can halt or slow breast cancers that need these hormones to grow.
How much of an impact can this have on your risk of breast and ovarian cancers? A significant one. If you have a BRCA mutation, prophylactic oophorectomy reduces your risk of breast cancer by about 50 percent if you're premenopausal, and it reduces your risk of ovarian cancer by 80 percent or more — regardless of your menopausal status. But if you don't have a BRCA mutation or a genetic predisposition based on a strong family history of breast or ovarian cancer, the drawbacks of this surgery probably outweigh the benefits.
Tubal ligation
A small incision is made in the abdomen and the fallopian tubes are either cut, tied or blocked. A hospital visit is required but normal sexual intercourse can normally resume within one week. A tubal ligation does not mean that the body will enter a menopausal state. The egg is still produced every month but because the fallopian tubes are cut off from the uterus, the egg dissolves in the tube. To arrange a tubal ligation, call your doctor for a recommended physician in the field.
Women with a high probability of developing ovarian cancer could reduce their risk by having their Fallopian tubes. Researchers studied women with a history of ovarian cancer and compared them with healthy women. It turned out that those who carried the BRCA 1 gene, meaning a high risk of ovarian cancer, had a 60 percent decrease in their risk if they had a tubal ligation.