Types of Cancer : Ovarian Cancer

Definition

Receiving a diagnosis of ovarian cancer is difficult and life changing. Fortunately, medical advances have made treatments more effective. Women diagnosed in the earliest stages have a five-year survival rate of nearly 93 percent, according to the American Cancer Society (ACS).

Until recently, ovarian cancer was known as a "silent killer" because it usually wasn't found until it had spread to other areas of your body. But new evidence shows that most women may have symptoms even in the early stages, and awareness of symptoms may hopefully lead to earlier detection.

Early detection is important; still, only about 20 percent of ovarian cancers are found before tumor growth has spread beyond the ovaries. Your chance of surviving ovarian cancer is better if the cancer is found early.

Symptoms

Symptoms of ovarian cancer are nonspecific and mimic those of many other more common conditions, including digestive and bladder disorders. A woman with ovarian cancer may be diagnosed with another condition before finally learning she has cancer. Common misdiagnoses include irritable bowel syndrome, stress and depression.

The key seems to be persistent or worsening signs and symptoms. With most digestive disorders, symptoms tend to come and go, or they occur in certain situations or after eating certain foods. With ovarian cancer, there's typically little fluctuation — symptoms are constant and gradually worsen.

Recent studies have shown that women with ovarian cancer are more likely than are other women to consistently experience the following symptoms:

• Abdominal pressure, fullness, swelling or bloating
• Urinary urgency
• Pelvic discomfort or pain

Additional signs and symptoms that women with ovarian cancer may experience include:

• Persistent indigestion, gas or nausea
• Unexplained changes in bowel habits, such as constipation
• Changes in bladder habits, including a frequent need to urinate
• Loss of appetite or quickly feeling full
• Increased abdominal girth or clothes fitting tighter around your waist
• Pain during intercourse (dyspareunia)
• A persistent lack of energy
• Low back pain
• Changes in menstruation

When to see a doctor
See your doctor if you have swelling, bloating, pressure or pain in your abdomen or pelvis that lasts for more than a few weeks. If you've already seen a doctor and received a diagnosis other than ovarian cancer, but you're not getting relief from the treatment, schedule a follow-up visit with your doctor or get a second opinion. Make sure that a pelvic exam is a part of your evaluation.

If you have a history of ovarian cancer or a strong history of breast cancer in your family, strongly consider seeing a doctor trained to detect and care for ovarian cancer patients so that you can talk about screening, genetic testing and treatment options while you are disease-free.

Causes

Women have two ovaries, one on either side of the uterus. The ovaries — each about the size of an almond — produce eggs (ova) as well as the female sex hormones estrogen and progesterone. An ovarian tumor is a growth of abnormal cells that may be either noncancerous (benign) or cancerous (malignant). Although benign tumors are made up of abnormal cells, these cells don't spread to other body tissues (metastasize). Ovarian cancer cells metastasize in one of two ways. Generally, they spread directly to adjacent tissue or organs in the pelvis and abdomen. They can also spread through your bloodstream or lymph channels to other parts of your body.

Three basic types of ovarian tumors exist, designated by where they form in the ovary. They include:

• Epithelial tumors. About 85 to 90 percent of ovarian cancers develop in the epithelium, the thin layer of tissue that covers the ovaries, according to the American Cancer Society.
• Germ cell tumors. These tumors occur in the egg-producing cells of the ovary and generally occur in younger women.
• Stromal tumors. These tumors develop in the estrogen- and progesterone-producing tissue that holds the ovary together.

The exact cause of ovarian cancer remains unknown. Some researchers believe it has to do with the tissue-repair process that follows the monthly release of an egg through a tiny tear in an ovarian follicle (ovulation) during a woman's reproductive years. The formation and division of new cells at the rupture site may set up a situation in which genetic errors occur. Others propose that the increased hormone levels before and during ovulation may stimulate the growth of abnormal cells.

Risk factors

Certain factors may increase your risk of ovarian cancer. Having one or more of these risk factors doesn't mean that you're sure to develop ovarian cancer, but your risk may be higher than that of the average woman. These risk factors include:

• Inherited gene mutations. While the vast majority of women who develop ovarian cancer don't have an inherited gene mutation, the most significant risk factor for ovarian cancer is having an inherited mutation in one of two genes called breast cancer gene 1 (BRCA1) and breast cancer gene 2 (BRCA2). These genes were originally identified in families with multiple cases of breast cancer, which is how they got their names, but people with these mutations also have a significantly increased risk of ovarian cancer.

  Women with the BRCA1 mutation have a 35 to 70 percent higher risk of ovarian cancer than do women without this mutation, and for women with a BRCA2 mutation, the risk is between 10 and 30 percent higher. For most women, the overall lifetime risk is about 1.5 percent, according to the ACS. You're at particularly high risk of carrying these types of mutations if you're of Ashkenazi Jewish descent.

  Another known genetic link involves an inherited syndrome called hereditary nonpolyposis colorectal cancer (HNPCC). Women in HNPCC families are at increased risk of cancers of the uterine lining (endometrium), colon, ovary and stomach. Risk of ovarian cancer associated with HNPCC is lower than is that of ovarian cancer associated with BRCA mutations.

• Family history. Sometimes, ovarian cancer occurs in more than one family member but isn't the result of any known inherited gene alteration. Having a family history of ovarian cancer increases your risk of the disease by 10 to 15 percent, according to the ACS.
• A history of breast cancer. If you've been diagnosed with breast cancer, your risk of ovarian cancer also is elevated.
• Age. Ovarian cancer most often develops after menopause. Your risk of ovarian cancer increases with age through your late 70s. Although most cases of ovarian cancer are diagnosed in postmenopausal women, the disease also occurs in premenopausal women.
• Childbearing status. Women who have had at least one pregnancy appear to have a lower risk of developing ovarian cancer. Similarly, the use of oral contraceptives appears to offer some protection against ovarian cancer.
• Infertility. If you've had trouble conceiving, you may be at increased risk. Although the link is poorly understood, studies indicate that infertility increases the risk of ovarian cancer, even without use of fertility drugs. Some research has also suggested that taking fertility drugs, such as clomiphene (Clomid), for more than one year may increase your risk of ovarian cancer, but it's not clear whether the increased risk actually comes from the drug or from the infertility.
• Hormone replacement therapy (HRT). Findings about the possible link between postmenopausal use of the hormones estrogen and progestin and risk of ovarian cancer have been inconsistent. However, a recent analysis of numerous studies, published in the journal Gynecologic Oncology, confirmed an association between HRT and ovarian cancer, particularly for those who took estrogen only. The risk appears to be highest among women who took HRT for more than five years.
• Obesity. Women who are obese have a greater risk of ovarian cancer. Obesity may also be linked to more-aggressive ovarian cancers, which can result in a shorter time to disease relapse and a decrease in the overall survival rate.
• Male hormones. The medication danazol, a male hormone (androgen), is used to treat endometriosis and has been linked to an increased risk of ovarian cancer. More study is needed to further define this association.

Preparing for your appointment

You're likely to start by first seeing your family doctor, general practitioner or a gynecologist. If your primary care physician suspects you have ovarian cancer, he or she may refer you to a specialist in female reproductive cancers (gynecologic oncologist), or you may ask for a referral yourself. A gynecologic oncologist is an obstetrician and gynecologist (OB-GYN) who has additional training in the diagnosis and treatment of ovarian and other gynecologic cancers.

Because appointments can be brief and there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, as well as any vitamins or supplements, that you're taking.
• Ask a family member or friend to join you, if possible. Sometimes it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions you want to ask your doctor.

Your time with your doctor may be limited, so preparing a list of questions before your appointment can help you make the most of your time together. List your questions from most important to least important in case time runs out. For ovarian cancer, some basic questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• Are there any other possible causes for my symptoms?
• What kinds of tests do I need?
• What type of ovarian cancer do I have?
• What types of treatments are available, and what kinds of side effects can I expect?
• What do you feel is the best course of action?
• What is my prognosis?
• If I still want to have children, what options are available to me?
• Will I have to stop working?
• I have these other health conditions. How can I best manage them together?
• Are there any restrictions that I need to follow?
• Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?

In addition to the questions that you've prepared, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Have your symptoms been continuous, or occasional?
• How severe are your symptoms?
• Does anything seem to improve your symptoms?
• Does anything appear to worsen your symptoms?
• Any first-degree relatives with ovarian or breast cancer? Other cancers in the family?

Tests and diagnosis

Because no standardized screening test exists to reliably detect ovarian cancer, doctors don't recommend screening for this type of cancer for most women. If you have a high risk of ovarian cancer, you need to have a careful discussion with your doctor about the risks and benefits of undergoing screening tests. While you may wonder what harm could come from a screening test, such tests can lead to unnecessary operations or other procedures that have significant side effects. Along with the potential complications of surgery, unnecessary procedures can cause anxiety, loss of work and substantial financial costs.

Several screening tests for ovarian cancer are commercially available, but none has been proved to lead to an early diagnosis that improves outcome. Additionally, these tests may lead to false-positives, which may subject women to unnecessary procedures and worry.

If your doctor suspects your symptoms suggest the presence of ovarian cancer, he or she may recommend one or more of the following tests to diagnose ovarian cancer:

• Pelvic examination. Your doctor examines your vagina, uterus, rectum and pelvis, including your ovaries, for masses or growths. If you've had your uterus removed (hysterectomy) but still have your ovaries, continue getting regular pelvic exams.
• Ultrasound. Ultrasound uses high-frequency sound waves to produce images of the inside of the body. Pelvic ultrasound provides a safe, noninvasive way to evaluate the size, shape and configuration of the ovaries. If a mass is found, however, ultrasound can't reliably differentiate a cancerous growth from one that's not cancerous. Ultrasound can also detect fluid in your abdominal cavity (ascites), a possible sign of ovarian cancer. Because ascites develops in many conditions other than ovarian cancer, however, its presence necessitates more testing.
• CA 125 blood test. CA 125 is a protein made by your body in response to many different conditions. Many women with ovarian cancer have abnormally high levels of CA 125 in their blood. However, a number of noncancerous conditions also cause elevated CA 125 levels, and many women with early-stage ovarian cancer have normal CA 125 levels. Because of this lack of specificity, the CA 125 test isn't used for routine screening in average-risk women and is of uncertain benefit in high-risk women.

Other diagnostic tests may include computerized tomography (CT) and magnetic resonance imaging (MRI), which both provide detailed, cross-sectional images of the inside of your body. Your doctor may also order a chest X-ray to determine if cancer has spread to the lungs or to the pleural space surrounding the lungs, where fluid can accumulate. If fluid is present, a needle may be inserted into the space to remove it. The fluid is then checked in the laboratory for cancer cells.

Confirming the diagnosis
If these tests suggest ovarian cancer, you'll need an operation to confirm the diagnosis. In a surgical procedure called laparotomy, a gynecologic oncologist makes an incision in your abdomen and explores your abdominal cavity to determine whether cancer is present. The surgeon may collect samples of abdominal fluid and remove an ovary for examination by a pathologist.

In certain cases, a less invasive surgical procedure called laparoscopy may be used. Laparoscopy requires only a couple of small incisions, through which a lighted instrument is inserted, along with small tools used to perform the surgery. Laparoscopy may be used if a surgeon wants to remove a tissue mass to determine whether it's cancerous before proceeding with a more invasive operation.

If ovarian cancer is confirmed, the surgeon and pathologist identify the type of tumor and determine whether the cancer has spread. This will help determine the stage of the disease. The surgeon usually will then need to extend the incision so that he or she can perform a more extensive operation to remove as much cancer as possible. It's important that this type of surgery be performed by a doctor specifically trained to treat gynecologic cancers.

Before you have a diagnostic operation, talk with your doctor about what your treatment options will be if you turn out to have a noncancerous abnormality on your ovary. If you're near or past menopause, your doctor may recommend removing both of your ovaries to decrease your risk of ovarian cancer.

Staging your cancer
Ovarian cancer is classified in stages I through IV, with stage I being the earliest and stage IV, the most advanced. Staging is determined at the time of surgical evaluation of the disease:

• Stage I. Ovarian cancer is confined to one or both ovaries.
• Stage II. Ovarian cancer has spread to other locations in the pelvis, such as the uterus or fallopian tubes.
• Stage III. Ovarian cancer has spread to the lining of the abdomen (peritoneum) or to the lymph nodes within the abdomen. This is the most common stage of disease identified at the time of diagnosis.
• Stage IV. Ovarian cancer has spread to organs beyond the abdomen.

Treatments and drugs

Treatment of ovarian cancer usually involves a combination of surgery and chemotherapy.

Surgery
In most cases, you'll want to have a gynecologic oncologist perform ovarian cancer surgery because they often have more training and experience with this type of operation.

Generally, women with ovarian cancer require an extensive operation that includes removing both ovaries, fallopian tubes, and the uterus as well as nearby lymph nodes and a fold of fatty abdominal tissue known as the omentum, where ovarian cancer often spreads.

During this procedure, your surgeon also removes as much cancer as possible from your abdomen (surgical debulking). Ideally, less than a total of 1 cubic centimeter of tumor matter remains in your abdominal cavity after surgery (optimal debulking). This may involve removing part of your intestines.

In addition, your surgeon will take samples of tissue and fluid from your abdomen to examine for cancer cells. This evaluation is critical in identifying the stage of your disease and determining if you need additional therapy.

If you want to preserve the option to have children and if your tumor is discovered early, your surgeon may be able to remove only the involved ovary and its fallopian tube. But, subsequent chemotherapy may cause infertility. However, in some cases, it is possible to successfully bear children after treatment. Be sure to discuss your desire to have children with your doctor.

Chemotherapy
After surgery, you'll most likely be treated with chemotherapy — drugs designed to kill any remaining cancer cells. The initial regimen for ovarian cancer includes the combination of carboplatin (Paraplatin) and paclitaxel (Taxol) injected into the bloodstream (intravenous administration). Clinical trials have found that this combination is effective, though researchers are continually looking for ways to improve on it.

A more intensive regimen has recently been shown to improve survival in women with advanced ovarian cancer by combining standard intravenous chemotherapy with chemotherapy injected directly into the abdominal cavity through a catheter placed at the time of the initial operation. This intra-abdominal infusion exposes hard-to-reach cancer cells to higher levels of chemotherapy than can be reached intravenously.

Side effects — including abdominal pain, nausea and vomiting — may leave many women unable to complete a full course of treatment or others to forego treatment entirely. But even an incomplete course of this treatment may help women live longer.

Other treatments being explored include new chemotherapy drugs, vaccines, gene therapy and immunotherapy, which boosts the immune system to help combat cancer. The newest option, if standard chemotherapy fails, is a drug called bevacizumab (Avastin). It works by disrupting the blood supply to the tumor, possibly causing it to shrink. The most serious side effect associated with bevacizumab is bowel perforation, which occurs in about 7 percent of people taking it.

Radiation
While a mainstay in the treatment of some other cancers, radiation generally isn't considered effective for ovarian cancer. Sometimes, your doctor may recommend external beam radiation therapy (EBCT) to treat the symptoms of advanced cancer.