Ovarian cancer is a disease in which, depending on the type and stage, malignant (cancerous) cells are found inside, near, or on the outer layer of the ovaries. An ovary is one of two small, almond-shaped organs located on each side of the uterus that store eggs, or germ cells, and produce the female hormones estrogen and progesterone. Ovarian cancer is the seventh most common cancer among women.
Types of Ovarian Cancer
Epithelial ovarian cancer is the most common and accounts for 85–89 percent of ovarian cancers. It forms on the surface of the ovary in the epithelial cells or from the fallopian tube. It ranks fourth in cancer deaths among women and causes more deaths than any other cancer of the female reproductive system.
On the other hand, germ cell cancer is an uncommon form of ovarian cancer, accounting for only about five percent of ovarian cancers. Germ cell cancers start in the cells that form the eggs in the ovaries. This cancer is usually found in adolescents and young women, and usually affects only one ovary.
Equally rare, stromal cell cancer starts in the cells that produce female hormones and hold the ovarian tissues together.
Familial breast-ovarian cancer syndrome is a common inherited condition that causes 15–20 percent of all ovarian cancers and five to ten percent of all breast cancers. The association is so common that it is now recommended that all women with epithelial ovarian cancer be tested for an inheritable BRCA mutation. This has implications for outcome, treatment, risk for breast cancer, and risk for family members. However, the implications of testing are complex enough that consultation with a genetic counselor is helpful.
Ovarian Cancer Symptoms
Historically, ovarian cancer was called the “silent killer” because symptoms were not thought to develop until the chance of cure was poor. However, recent studies have shown this term is untrue and that the following signs are much more likely to occur in women with ovarian cancer than in women in the general population, even in patients with early-stage disease.
These symptoms include:
- Pelvic or abdominal pain
- Difficulty eating or feeling full quickly
- Urinary symptoms (urgency or frequency)
Women with ovarian cancer report that symptoms are persistent and represent a change from normal for their bodies. The frequency or number of ovarian cancer signs are key factors in the diagnosis of ovarian cancer. Several studies show that even early-stage ovarian cancer can produce these symptoms.
Women who have these symptoms almost daily for more than a few weeks should see their doctor, preferably a gynecologist. Prompt medical evaluation may lead to detection at the earliest possible stage of the disease. Early-stage diagnosis is associated with an improved prognosis. Several other symptoms have been commonly reported by women with ovarian cancer. These symptoms include fatigue, indigestion, back pain, pain with intercourse, constipation, and menstrual irregularities. However, these other symptoms are not as useful in identifying ovarian cancer because they are also found in equal frequency in women in the general population who do not have ovarian cancer.
Stages of Ovarian Cancer
Cancer is limited to one or both ovaries.
Cancer is limited to one ovary and the tumor is confined to the inside of the ovary. No ascites containing malignant cells is present, and the surface of the tumor has not ruptured.
Cancer is limited to both ovaries without any tumors on the ovaries’ outer surfaces. No ascites containing malignant cells is present, and the surface of the tumor has not ruptured.
The tumor is classified as either Stage IA or IB and one or more of the following conditions exist:
- a tumor on the outer surface of one or both ovaries;
- at least one ruptured tumor;
- ascites or abdominal (peritoneal) washings containing malignant cells.
The tumor involves one or both ovaries and extends to other pelvic structures.
The cancer has extended to and/or involves the uterus and/or the fallopian tubes.
The cancer has extended to the bladder or rectum.
The tumor is classified as either Stage IIA or IIB and one or more of the following conditions exist:
- a tumor on the outer surface of one or both ovaries;
- at least one ruptured tumor;
- ascites containing malignant cells or abdominal (peritoneal) washings containing malignant cells.
The tumor involves one or both ovaries, and one or both of the following exist:
- The cancer has spread beyond the pelvis to the lining of the abdomen;
- The cancer has spread to the lymph nodes. The tumor is limited to the true pelvis but with histologically-proven malignant extension to the small bowel or omentum (peritoneum fold).
The tumor is in one or both of the ovaries. While surgeons cannot see cancer in the abdomen, and the cancer has not spread to the lymph nodes, biopsies checked under a microscope reveal tiny deposits of cancer in the abdominal (peritoneal) surfaces.
The tumor is in one or both ovaries, and deposits of cancer are present in the abdomen that are large enough for the surgeon to see but do not exceed 2cm in diameter. The cancer has not spread to the lymph nodes.
The tumor is visible in one or both ovaries, and one or both of the following conditions exists:
- the cancer has spread to lymph nodes;
- the deposits of cancer exceed 2cm in diameter and are found in the abdomen.
Growth of the cancer involves one or both ovaries and distant metastases to the liver or lungs have occurred.
Finding Ovarian Cancer cells in the excess fluid accumulated around the lungs (pleural fluid) also shows evidence of Stage IV.
Ovarian Cancer Treatment and Side Effects
Ovarian cancer is most often treated with surgery and chemotherapy. Whether surgery or chemotherapy is used first will depend on several factors specific to your disease. Only rarely is radiation therapy used. It is important to distinguish between early-stage ovarian cancer and advanced disease because the treatment approaches are different.
All treatments for ovarian cancer have side effects. Most side effects can be managed or avoided. Treatments may affect unexpected parts of your life, including your function at work, home, intimate relationships, and deeply personal thoughts and feelings.
Before beginning treatment, it is important to learn about the possible side effects, and talk with your treatment team members about your feelings or concerns. They can prepare you for what to expect and tell you which side effects should be reported to them immediately. They can also help you find ways to manage the side effects that you experience.
Understanding the Goals of Treatment
As you begin your treatment, make sure that you understand what to expect. Is this for a cure? What are the chances of a cure? If there is no cure, will the treatment make me live better or longer? It is very important to understand the truth about what to expect from the treatment—and what are the potential side effects, expenses, etc.—so that you can make the best decisions for yourself and the life you want to lead.
Ovarian Cancer Treatment Options
Surgery is usually the first step in treating ovarian cancer and it should be performed by a gynecologic oncologist. Most surgery is performed using a procedure called a laparotomy during which the surgeon makes a long cut in the wall of the abdomen. Occasionally, early-stage ovarian cancer can be managed by laparoscopic surgery whereby multiple (1/2” to 3/4”) small incisions are made in the belly button or lower abdomen. Laparoscopic surgery sometimes can also be performed in women with advanced ovarian cancer.
If ovarian cancer is found, the gynecologic oncologist usually performs the following procedures:
Salpingo-oophorectomy: both ovaries and fallopian tubes are removed.
Hysterectomy: the uterus is removed.
Staging procedure: including omentectomy, lymph node removal.
Debulking: removal of any additional visible disease.
For staging, the omentum, a fatty pad of tissue that covers the intestines, is removed along with nearby lymph nodes and multiple tiny samples of tissues from the pelvis and abdomen.
If the cancer has spread, the gynecologic oncologist removes as much cancer as possible. This is called “debulking” surgery. Often this will involve extensive surgery, including removal of portions of the small or large intestine and removal of tumor from the liver, diaphragm, and pelvis. Removal of as much tumor as possible is one of the most important factors affecting cure rates.
If you have early stage I cancer and still hope to get pregnant, it may be possible to only remove one ovary and fallopian tube. Your future pregnancy wishes should be discussed with your gynecologic oncologist before surgery.
Goals of surgery
It is important to understand that the goals of surgery are to remove as much cancer as possible – with the best outcome if the surgeon can remove all visible cancer. In some cases, your surgeon may want to start with a laparoscopy to look inside to determine if the cancer can be optimally removed. In this case, the surgeon may proceed with the full operation and “debulking” or stop and plan for neoadjuvant chemotherapy to shrink the tumor(s) so that they can be removed after several treatments of chemotherapy.
Side effects of surgery
Some discomfort is common after surgery. It often can be controlled with medicine. Tell your treatment team if you are experiencing pain. Talk to your doctor if you are experiencing any other possible side effects, such as:
- Nausea and vomiting
- Infection, fever
- Wound problem
- Fullness due to fluid in the abdomen
- Shortness of breath due to fluid around the lungs
- Swelling caused by lymphedema, usually in the legs
- Blood clots
- Difficulty urinating or constipation
Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy for ovarian cancer is usually given intravenously (injected into a vein). You may be treated in the doctor’s office or the outpatient part of a hospital.
The drugs travel through the bloodstream to reach all parts of the body. This is why chemotherapy can be effective in treating ovarian cancer that has spread beyond the ovaries. However, the same drugs that kill cancer cells may also damage healthy cells, leading to side effects. Chemotherapy is usually given in cycles. Periods of chemotherapy treatment are alternated with rest periods when no chemotherapy is given. Most women with ovarian cancer receive chemotherapy for about 6 months following their surgery. In some cases, it may be appropriate to continue chemotherapy for a longer period of time to reduce the chance of the cancer returning.
There is another way to deliver chemotherapy, called intraperitoneal (IP) chemotherapy. With IP chemotherapy, the medications are injected directly into the abdominal cavity in hopes of delivering a large dose directly to the tumor location. Usually, some of the chemotherapy is administered into the belly and some is still administered in the vein. Your surgeon may talk to you about placing a special catheter in your belly at the time of your operation if he/she feels that you could benefit from IP chemotherapy.
Intraperitoneal chemotherapy is recommended for women with stage III ovarian cancer in whom all of the tumor spots bigger than 1 centimeter were removed with surgery. Recent studies have shown that while IP chemotherapy has more short-term toxicity, it is associated with a longer survival rate. It is important for you to talk with your team about the pros and cons of this approach.
Occasionally, cancers will be advanced at initial diagnosis and your gynecologic oncologist may feel that surgery is unlikely to be as effective as desired, or that immediate surgery will be too difficult for you to tolerate. In this situation, chemotherapy treatments can be given to shrink the tumor before surgery takes place. This is called neoadjuvant chemotherapy. Once there has been shrinkage of the tumor and your physical condition is improving, surgery is performed, usually followed by more chemotherapy. Since most women with ovarian cancer present with advanced-stage disease, which is determined by a clinical exam and imaging, a biopsy may be needed if neoadjuvant chemotherapy is going to be considered.
Side effects of chemotherapy
Each person responds to chemotherapy differently. Some people may have very few side effects while others experience several. Most side effects are temporary. They include:
- Loss of appetite
- Mouth sores
- Increased chance of infection
- Bleeding or bruising easily
- Hair loss
- Neuropathy (weakness, numbness, and pain from nerve damage)
- “Chemo brain” (memory lapses, problems with concentration)
Radiation therapy (also called radiotherapy) uses high-energy x-rays, or other types of radiation, to kill cancer cells or stop them from growing. Radiation therapy is not usually part of the first treatment plan for women with ovarian cancer, but may be used if the tumor returns.
Side effects of radiation
The side effects of radiation therapy depend on the dose used and the part of the body being treated. Common side effects include:
- Dry, reddened skin in the treated area
- Discomfort when urinating
- Narrowing of the vagina
Most of these side effects are temporary. Be sure to talk with your treatment team members about any side effects you experience. They can help you find ways to manage them.
A few types of ovarian cancer need hormones to grow. In these cases, hormone therapy may be a treatment option. Hormone therapy removes female hormones or blocks their action as a way of preventing ovarian cancer cells from getting or using the hormones they may need to grow. Hormone therapy is usually taken as a pill but can be given as a shot.
Side effects of hormone therapy
The side effects depend on the type of hormones being used. Some women retain fluid and have a change in appetite, or have hot flashes.
There are many new treatments that are targeting specific mechanisms of tumor growth. For example, there are drugs that can block new blood vessel formation or target specific tumor enzymes to inhibit tumor growth. Many of these new agents are being investigated in clinical trials. Because these drugs block pathways that are more active in tumor cells, they are not as damaging to normal cells. Sometimes these targeted therapies are combined with chemotherapy to try to make the chemotherapy more effective. For patients, this often means fewer serious side effects, but targeted therapy drugs have their own unique side effects, which will be discussed by your team.
Follow Up After Treatment
- In general, women receive follow up exams (including a pelvic exam) every 3 to 4 months for 3 years, and then every 6 months. In addition, CA 125 and imaging studies such as x-rays, CT scans, or MRIs may be periodically performed, especially if you have any new pains or symptoms.
- Recurrences are often diagnosed when the CA 125 level begins to rise, or new masses are found on imaging studies or with examination. A biopsy may be required to be certain a lesion is a recurrent tumor.
- If ovarian cancer recurs, there are several options for treatment. These include repeating surgery, re-treatment with the same chemotherapy given initially, treatment with a different type of agent (chemotherapy, hormonal, or targeted therapy), and sometimes radiation. As each recurrence will be different, it is important to discuss your individual situation with your team. It is also important to investigate whether there is a clinical trial that is appropriate for you. Don’t be afraid to seek a second opinion.
- A recent advance in ovarian cancer treatment is the successful use of poly ADP ribose polymerase (PARP) inhibitors. This category of drugs is especially effective in patients with BRCA1 and BRCA2 mutations. Currently only one PARP inhibitor has received FDA approval for ovarian cancer patients who are BRCA1/2 positive. Several other PARP inhibitors are undergoing clinical trials. This advance underscores the strong recommendation that all women with ovarian cancer be tested for the BRCA1/2 mutations. This is important information for family members and can guide treatment options for patients.
Importance of participation in clinical trials
There are many ongoing clinical trials studying new and better ways to treat ovarian cancer. Many treatment options are available today because women diagnosed with ovarian cancer were willing to participate in prior clinical trials.