In general, ovarian tumors are named according to the kind of cells the tumor started from and whether the tumor is benign or cancerous. There are three main types of ovarian tumors. Epithelial tumors start from the cells that cover the outer surface of the ovary. Germ cell tumors start from the cells which produce the ova or eggs. Stromal tumors start from connective tissue cells which hold the ovary together and produce the female hormones, estrogen and progesterone. This grouping is based on how the cells appear under a microscope.
Epithelial ovarian tumors: Most epithelial ovarian tumors are benign. There are several types of benign epithelial tumors, including serous adenomas, mucinous adenomas, and Brenner tumors. Cancerous epithelial tumors are carcinomas. Some ovarian epithelial tumors have an appearance under the microscope which does not clearly indicate that they are cancerous -- these are called atypical proliferating tumors. They are also referred to as borderline tumors or tumors of low malignant potential (LMP tumors). Epithelial ovarian carcinomas (EOC) account for 85%-90% of cancers of the ovaries. The cells which form EOC may have several forms which can be recognized under the microscope. In this way, EOC are divided into serous, mucinous, endometrioid, and clear cell types. Undifferentiated EOC lack distinguishing features of any of these four subtypes and tend to grow and spread more quickly. In addition to their classification by cell type, EOC are given a grade and stage. The grade is on a scale of 1, 2, or 3. Grade 1 EOC more closely resemble normal tissue and tend to have a better prognosis (outlook). Grade 3 EOC less closely resemble normal tissues and usually have a worse outlook. The tumor stage describes how far the tumor has spread from where it started in the ovary. Staging is explained in detail in a later section.
- Germ cell tumors: This type of tumor starts in cells which normally form the ova or eggs. These germ cells may form several types of tumors. Most germ cell tumors are benign, although some are cancerous and may be life-threatening. The most common germ cell tumors are described below:
- Teratoma - This germ cell tumor has a benign form called mature teratoma and a cancerous form called immature teratoma. The mature teratoma is by far the most common germ cell tumor, usually affecting women of reproductive age (teens through forties). It is often called a " dermoid cyst " because its lining resembles skin. These tumors contain a variety of benign tissues resembling adult skin, respiratory passages, bone, teeth, etc. Surgical removal of the cyst is curative. Immature teratomas occur in girls and young women, usually younger than 18. These rare cancers resemble embryonic or fetal tissues such as connective tissue, respiratory passages, and brain. When they are localized to the ovary and the immaturity is not prominent (grade 1 immature teratoma), they are cured by surgical removal of the ovary. When they have spread beyond the ovary and/or much of the tumor has a very immature appearance (grade 2 or 3 immature teratomas), chemotherapy is recommended in addition to surgical removal of the ovary.
- Dysgerminoma - Although this is the most common ovarian cancer of germ cells, it represents only 2% of all ovarian cancers. It usually affects women in their teens and twenties. Although dysgerminomas are considered malignant (cancerous), most do not grow or spread very rapidly. When they are limited to the ovary, over 95% are cured by surgical removal of the ovary, without any treatment. Even when the tumor has spread further, the combination of surgery and chemotherapy is effective in more than 80% of patients.
- Endodermal Sinus Tumor (Yolk Sac Tumor) and Choriocarcinoma - These are very rare tumors in girls and young women. These tumors tend to grow and spread rapidly but are often sensitive to chemotherapy. Choriocarcinomas more commonly start in the placenta (during pregnancy) rather than in the ovary. Placental choriocarcinomas are usually quite responsive to chemotherapy.
- Stromal tumors: These tumors account for about 5% of ovarian tumors. More than half are noted in women after the age of 50, but some occur in young girls. Some, but not all, of these tumors produce female hormones or, less commonly, male hormones. They can cause vaginal bleeding to resume after menopause, or can cause menstrual periods and breast development in young girls. If male hormones are produced, the tumors can disrupt normal periods and cause facial and body hair to grow. The most common types among the rare class of stromal tumors are granulosa-theca tumors, and Sertoli-Leydig cell tumors. These tumors are quite rare and are usually considered low grade cancers. Thecomas and fibromas are benign.
DO WE KNOW WHAT CAUSES OVARIAN CANCER?
No. We do not yet know what causes ovarian cancer, but we do know some risk factors for the most common type of ovarian cancer -- epithelial ovarian cancer. There is a great deal of research underway to learn more about every aspect of the disease.
During the past few years scientists have learned much about how certain genes inherited from a woman's parents can greatly increase her ovarian cancer risk. These include the BRCA1 and BRCA2 genes, and several genes related to the Lynch II syndrome. However, these inherited risks explain only about 5% of ovarian cancers.
Other types of research have found certain reproductive and dietary factors which increase ovarian cancer risk. These include having no children or having your first child after age 30, and possibly, eating a high-fat diet. However, the great majority of ovarian cancers are not explained by any known risk factor.
It is important to remember that risk factors increase the odds of getting a disease but do not guarantee it will occur. For example, we know that, on the average, women who have their first child after 30 have a slightly greater risk of developing ovarian cancer than women who had their first child before 30. This does not mean that all woman with ovarian cancer who had their first child at 30 could have prevented the cancer by having that child a year earlier.
The American Cancer Society is funding major research into the causes, prevention, and treatment of ovarian cancer.
HOW IS OVARIAN CANCER TREATED?
After the diagnostic tests are done, your cancer care team will recommend a treatment strategy. Consider the options without feeling rushed. If there is anything you do not understand, ask to have it explained again. The choice of treatment depends largely on the type of cancer and stage of the disease at discovery. In patients who do not have surgery as their initial treatment, the exact stage may not be determined. Treatment then is based on other available information. Other factors could play a part in choosing the best treatment plan. This might include your age, whether you plan to have children, and other personal considerations. Be sure you understand all the risks and side effects of the various therapies before making a decision about treatment.
Age alone is not a determining factor since several studies have shown that older women tolerate ovarian cancer treatments well.
Treatment options for ovarian cancer
Surgery: How much surgery you have depends on how far your cancer has spread and on your general health. For women of childbearing age who have certain kinds of tumors and whose cancer is in the early stage, an effort will be made to treat your disease without removing both ovaries and the uterus. There are several operations used to treat ovarian cancer. The medical vocabulary for these operations is based on the Greek or Latin medical names of the organs they remove. The medical name of an operation that removes something usually ends with "-ectomy." So, removing the uterus is a hysterectomy, removing the omentum is an omentectomy, removing lymph nodes is a lymphadenectomy (also called lymph node biopsy or dissection). Because there are two ovaries and two fallopian tubes, we must distinguish between removing one or both. Removing one ovary is a unilateral (one side) oophorectomy and removing both is a bilateral (two side) oophorectomy. Likewise, removing one or two fallopian tubes is a unilateral salpingectomy or bilateral salpingectomy. Often, an operation removes several organs. For example removing both ovaries and fallopian tubes is a bilateral salpingooophorectomy. This partial list of the names of operations should help you in understanding information you may read about ovarian cancer and in discussing your cancer with your health care providers. However, don't be afraid to ask your cancer care team to explain your condition and recommend treatments in simple, nonmedical terms.
Side effects of surgery: Removal of both ovaries and/or the uterus means that you will not be able to become pregnant. It also means that you will go into menopause if you have not done so already.
- Chemotherapy: Chemotherapy uses anticancer drugs that are given into a vein or by mouth. These drugs enter the bloodstream and reach all areas of the body, making this treatment especially useful for cancer that has spread beyond the ovaries. If this treatment is chosen, you will probably receive a combination of drugs. Combination chemotherapy is generally more effective in preventing return of ovarian cancer than one drug alone. These drugs kill cancer cells but can also damage some normal cells. Therefore, careful attention is given to avoiding or minimizing side effects.
Side effects of chemotherapy: The side effects of chemotherapy depend on the type of drugs, the amount taken, and the length of time you are treated. They include:
- Upset stomach and vomiting (new medications given together with the chemotherapy can prevent these unpleasant side effects)
- Loss or increase of appetite
- Temporary loss of hair (alopecia)
- Mouth or vaginal sores
- Increased chance of infections, bleeding, or anemia (low redblood cells)
- Changes in the menstrual cycle
- Premature menopause
- Infertility (inability to become pregnant)
- Acute myeloid leukemia (AML): A few of the anticancer drugs may rarely cause this life-threatening disorder of white blood cells. Most of the offending drugs are known, and your health care team will discuss this possibility with you. The small chance that any of these drugs will cause leukemia is offset by their positive effects against cancer.
Most of these side effects of chemotherapy (except premature menopause, infertility and AML) stop when the treatment is over. If you are taking chemotherapy and have side effects, there are remedies for many of them. Be sure to talk with your cancer care team about any side effects you are experiencing.
Radiation therapy: Radiation therapy uses high energy x-rays to kill cancer cells. These x-rays may be given externally in a procedure that is much like having a diagnostic x-ray. It also may be given as an implant of radioactive materials near the tumor area in the abdominal cavity. In this country, radiation therapy is only rarely used for ovarian cancer.
Side effects of radiation therapy: The skin in the treated area may look and feel sunburned. This gradually fades into a tanned look, returning to a normal appearance in six to 12 months. Many women also complain of tiredness, upset stomach, or loose bowels. If you are having side effects from radiation, discuss them with your cancer care team. There may be things you can do to obtain relief.
Clinical Trials: Clinical trials are scientific studies of promising new treatments. Ask your cancer care team about clinical trials for which you may qualify. You can obtain a current list of these trials from the National Cancer Institute's Cancer Information Service by calling 1-800-4-CANCER.
Treatment for epithelial ovarian cancers by stage
From 85% to 90% of all ovarian cancers are epithelial ovarian cancers. This type of cancer starts in the epithelium, or covering of the ovary. Current treatment by stage of the cancer follows:
- Stages IA and IB: For both stages, surgery is the treatment of choice. If the laboratory results indicate a grade 1 or grade 2 cancer (meaning the cancer has some similarities to normal glandular tissue) surgery alone may be enough. The surgery can include a hysterectomy (removal of the uterus, or womb), salpingectomy (removal of the fallopian tubes), oophorectomy (removal of both ovaries) and omentectomy (removal of part of the omentum, which is fatty tissue from the upper part of the abdominal cavity near the stomach and intestines). During surgery, biopsies (tissue samples) of organs, omentum, lymph nodes and the lining surfaces of the pelvic and abdominal cavities may be collected and sent to the laboratory for microscopic examination.
- Stage IC: For the stage IC or grade III tumor, surgery is the treatment of choice as described for stages IA and IB. Chemotherapy likely will be used, including drugs that contain platinum such as cisplatin or carboplatin. Radiation also may be given in the form of P32 solution (a radioactive liquid) inserted into the peritoneal cavity.
- Stage II (including IIA, IIB, IIC): Not many tumors are diagnosed at this stage. In such cases, the same type of surgery as described for stage I is performed. Additional treatment will consist of combination chemotherapy or, less often, radiation therapy.
- Stage III: For stages IIIA, IIIB, IIIC, the options are the same. The uterus, fallopian tubes, ovaries, and omentum (fatty tissue from the upper abdomen near the stomach and intestines) are removed. The tumor also will be "debulked." This means that its size will be reduced as much as is possible. The smaller the remaining tumor, the better the outlook for the patient's future. After surgery, combination chemotherapy may be used.
After surgery, during chemotherapy, and after chemotherapy, blood tests will be given to determine if you have normal levels of a tumor marker called CA-125. If CA-125 levels and imaging studies (such as CT scans or sonograms) are normal, your cancer care team may want to do a " second look " surgery (laparoscopy and/or laparotomy). For laparoscopy, a small opening is made below the navel and a slender tube with a light is placed so the doctor can inspect the abdominal cavity to see how successful treatment has been. Laparotomy requires an incision or surgical opening long enough to allow your surgeon to look inside the pelvis and abdomen and take biopsy samples. Based on the results of the " second-look " surgery, your cancer care team can decide whether more treatment is needed. (Second-look operations are not a standard part of ovarian cancer care but are usually performed as part of clinical trials.)
- Stage IV: For this stage, the same type of surgery as described for stage III may be performed. Combination chemotherapy with a platinum regimen may be used. As with stage III, second-look surgery may rarely be considered.
Recurrent or persistent ovarian cancer: Recurrent tumors are those that reappear after initial treatment. Persistent tumors are those that never disappeared even after treatment. For either of these types of ovarian cancer, a clinical trial for new treatments may provide important advantages. Ask your cancer care team for information on suitable trials for your type of cancer. If epithelial ovarian cancer recurs after initial treatment over a period of months or years, you may be offered additional surgery, followed by combination chemotherapy. Follow-up treatment like this is usually less successful than the initial treatment. However, if the initial disease-free period was long (a few years), there may be a good response to a second course of treatment.
Treatment for atypical proliferating epithelial tumors (" epithelial tumors of low malignant potential " or " borderline tumors ")
The ovary with the tumor and the fallopian tube on that side are usually removed. In certain cases just the ovarian cyst containing the tumor is removed (ovarian cystectomy). If there is no cancer seen beyond the one involved ovary and if the patient may want to become pregnant in the future, no further surgery is done at that time. If the patient is not interested in remaining fertile, both ovaries and fallopian tubes as well as the uterus are removed. If the tumor is in more advanced stages, it is debulked as completely as possible. Chemotherapy and radiotherapy are not generally used in the initial treatment of these tumors. If this tumor comes back after initial surgery, chemotherapy and radiotherapy may be considered.
Treatment for germ cell tumors of the ovary
Germ cell tumors of the ovary develop from the cells which normally would form the ova or eggs. These tumors tend to occur in girls and young women. Patients with benign (noncancerous) germ cell tumors such as mature teratomas (dermoid cysts) are cured by removal of a portion of the ovary (ovarian cystectomy) containing the tumor or rarely by removal of the ovary. It is a good idea to consult with a gynecologic oncologist for the treatment of malignant germ cell tumors.
All stages of germ cell ovarian cancer (except for stage IA dysgerminoma and stage I, grade I immature teratoma): Most types and stages of germ cell cancers of the ovary are treated the same, with a few important exceptions. In order to identify these special cases, precise classification of the tumor and attention to staging is needed.
- Surgery: If only one ovary is involved (stage IA) and you want to become pregnant later, only the involved ovary and the fallopian tube on that side are removed. The uterus and the ovary and tube on the opposite side are not taken out. On the other hand, if future pregnancy is not important to you, the uterus, both ovaries, and both fallopian tubes may be removed.
If cancer involves both ovaries (stage IB), both ovaries and both fallopian tubes will be removed. The uterus may be removed if childbearing is complete. However, the uterus can be left to allow future pregnancy through the use of in-vitro fertilization. If cancer has spread beyond the ovaries (stage IC and higher) the involved ovary and tube will be removed and debulking may be done (removing as much cancer as possible without damaging or removing essential organs).
- Chemotherapy: With the exception of patients with stage I, grade 1 immature teratoma, and some patients with stage IA dysgerminoma, all patients with germ cell cancers receive combination chemotherapy. A frequently used combination chemotherapy treatment is called BEP, combining three drugs called Bleomycin, Etoposide, and Platinol (cisplatin). However, other drug combinations may be used, particularly as part of a clinical trial or for treatment of cancer which has recurred (come back) after initial treatments.
- Radiation therapy: In the past, radiation therapy was often used for treating dysgerminomas. However, results with current combination chemotherapy are as good or better. For younger women who want to keep the option of future pregnancy and who have had only one ovary removed, chemotherapy is less damaging to the remaining ovary and less likely to cause difficulty in becoming pregnant. For these reasons, radiation therapy is rarely used as the main treatment for dysgerminoma.
In some situations, such as cancer recurrence, radiation rarely may be given in addition to chemotherapy.
- Stage IA dysgerminoma: If careful staging has found that dysgerminoma is limited to the inside of one ovary, the patient may be treated by removing only that ovary and the fallopian tube on the same side, without chemotherapy after surgery. This approach requires close follow up so that any recurrence can be found early and treated. The advantage of this approach is that the majority of patients in this stage will not have recurrence of their cancer and will not need to have any chemotherapy.
- Stage I, Grade 1 immature teratoma: A grade 1 immature teratoma is composed mostly of noncancerous tissue, with only a few cancerous areas seen under the microscope which are immature (looking like fetal organs). These tumors rarely come back after being removed. If careful staging has determined that a grade 1 immature teratoma is limited to one or both ovaries, the patient may be treated by removing the ovary or ovaries containing the cancer and the fallopian tube or tubes, without chemotherapy after surgery.
- Recurrent or persistent germ cell tumors: Recurrent tumors are those that reappear after initial treatment. Persistent tumors are those that never disappeared even after treatment. For either of these types of ovarian cancer, a clinical trial for new treatments may provide important advantages. Ask your cancer care team for information on suitable trials for your type of cancer.
Treatment for recurrent or persistent germ cell tumors may include chemotherapy or, rarely, radiation therapy. In chemotherapy, a combination of drugs is used, sometimes referred to as VIP, including Vinblastine, Ifosphamide, and Platinol. BEP chemotherapy may be used if the patient has never received this combination of drugs.
Treatment for stromal tumors of the ovary
- Stromal tumors: Stromal tumors (starting from connective tissue cells which hold the ovary together and produce the female hormones, estrogen and progesterone) are less common than epithelial or germ cell tumors. Most stromal tumors are benign (noncancerous) and are treated by surgical removal of the ovary containing the tumor. Malignant (cancerous) stromal tumors are less common. They are initially treated by removing the cancer as completely as possible. It they recur, chemotherapy may be used.
WHAT SHOULD YOU ASK YOUR PHYSICIAN ABOUT OVARIAN CANCER?
It is important for you to have frank, open discussions with your cancer care team. They want to answer all of your questions, no matter how trivial you might think they are. Here are some questions to consider:
- What type of cancer do I have?
- Has my cancer spread beyond the primary site?
- What is the stage of my cancer and what does that mean in my case?
- What treatments are appropriate for me? What do you recommend? Why?
- What are the risks or side effects that I should expect?
- What are the chances my cancer will recur (come back) with the treatment programs we have discussed?
- What should I do to be ready for treatment?
- Should I follow a special diet?
- Will I be able to have children after my treatment?
- What is my expected prognosis, based on my cancer as you view it?
- Where can I get a wig?
- What do I tell my children, husband, parents, and other family members?
In addition to these sample questions, be sure to write down some of your own. For instance, you might want specific information about anticipated recovery times so you can plan your work schedule. Or, you may want to ask about second opinions or about experimental programs or clinical trials for which you may qualify.
WHAT WILL HAPPEN AFTER TREATMENT FOR OVARIAN CANCER?
Each type of treatment for ovarian cancer could have side effects that may last from a few months to many years. A woman may be able to hasten her recovery by being aware of those side effects before treatment and by taking steps to minimize them and shorten their duration where possible after treatment.
From the start, you must keep in mind that you must deal with your own individual physical and emotional factors. In the process of deciding what kind of treatment to have, for instance, you will find it helpful to discuss options with your family and friends, as well as with your primary care physician and nurse. At every step of the way in pre-treatment, treatment, and recovery, you should talk with your cancer care team about side effects, ways to make them easier to endure, and the general outlook, or prognosis, for your case. They want to answer your questions, so ask them!
Your body is as unique as your personality and your fingerprints. Scientists can determine certain facts about tumors and drugs, and doctors can use a variety of treatments to eliminate the cancer. But no one can say precisely how you will respond to cancer or its treatment.
You may have special strengths such as a healthy immune system, a history of excellent nutrition, a strong family support system, a deep faith. All of these can make a difference in how you respond to cancer. In fact, behavioral scientists have recently found that women who took advantage of a social support system, such as a cancer support group, survived with a better quality of life.
From the scientific point of view, the prognosis, or outlook, for any ovarian cancer is determined by a number of factors. The most important is its stage, or how far the cancer has spread beyond the ovary. Other factors that influence survival rate include the size of the tumor, the type of tumor cells and their grade (degree of maturity as seen under a microscope).
An important part of your treatment plan is a specific schedule of follow-up visits after surgery, chemotherapy, or radiation therapy to be sure what, if any, additional treatment is necessary.
Blood tests may be needed periodically to help with early detection of recurrence. These tests will check the levels of CA 125, a tumor marker for epithelial ovarian cancer, or, in the case of some germ cell tumors, for levels of alpha fetoprotein (AFP) and human chorionic gonadotropin (HCG) in the blood.
Follow-up also may involve procedures such as x-rays, CT scans, ultrasound studies, or magnetic resonance imaging. There also may be biopsies to get tissue samples for laboratory microscopic evaluation and, in some circumstances, " second look " surgery.
You can help in your own recovery from cancer by making healthy lifestyle choices.
If you use tobacco, stop now. Quitting will improve your overall health and the full return of the sense of smell may help you enjoy a healthy diet during recovery.
If you use alcohol, limit how much you drink. Have no more than one or two drinks per day.
Good nutrition can help you get better after treatment. Eat a nutritious diet. Researchers are also finding increasing evidence of the importance of nutrition in the prevention of cancer. Eat more fruits, vegetables, whole grains, and high-fiber foods. Eat less fats, especially animal fat.
Exercise once you are well.
If you are in therapy for cancer, be aware of the battle that is going on in your body. Radiation therapy and chemotherapy add to the fatigue caused by the disease itself. Give your body all the rest it needs so that you will feel better as time goes on.
A cancer diagnosis and its treatment is a major life challenge, with an impact on you and everyone who cares for you. Before you get to the point where you feel overwhelmed, consider attending a meeting of a local support group. If you need individual assistance in other ways, contact your hospital's social service department or your local Division of the American Cancer Society for help in contacting counselors or other services.
Finally, your cancer care team is always ready to help you understand your disease and treatment to answer your questions about what you can do to improve your outlook for the future.