Childhood Ovarian Cancer Treatment (PDQ®): Treatment - Patient Information [NCI]

This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.

General Information about Childhood Ovarian Cancer

Ovarian cancer is a disease in which malignant (cancer) cells form in the tissues of the ovary.

The ovaries are a pair of organs in the female reproductive system. They are located in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond in an adult woman. The ovaries produce eggs and female hormones (chemicals that control the way certain cells or organs function).

Anatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium.

Most ovarian tumors in children are benign (not cancer). They occur most often in females aged 15 to 19 years.

There are several types of malignant ovarian tumors.

The types of malignant ovarian tumors include the following:

Germ cell tumors: Tumors that start in egg cells in females. These are the most common ovarian tumors in girls. (See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on ovarian germ cell tumors.)
Epithelial tumors: Tumors that start in the tissue covering the ovary. These are the second most common ovarian tumors in girls. Ovarian epithelial cancer is usually found at an early stage in children and is easier to treat than in adult patients.
Stromal tumors: Tumors that begin in stromal cells, which make up tissues that surround and support the ovaries. Juvenile granulosa cell tumors and Sertoli-Leydig cell tumors are two types of stromal tumors.
Small cell carcinoma of the ovary: Cancer that begins in the ovary and may have spread to the abdomen, pelvis, or other parts of the body. This type of ovarian cancer is fast growing and has a poor prognosis.

This summary is about ovarian non-germ cell tumors (epithelial tumors, stromal tumors, and small cell carcinoma of the ovary).

Having Ollier disease or certain syndromes may increase the risk of ovarian cancer.

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.

The risk of ovarian cancer is increased by having one of the following conditions:

Ollier disease (a disorder that causes abnormal growth of cartilage at the end of long bones).
Maffucci syndrome (a disorder that causes abnormal growth of cartilage at the end of long bones and of blood vessels in the skin).
Peutz-Jeghers syndrome (a disorder that causes polyps to form in the intestines and dark spots to form on the mouth and fingers).
Familial pleuropulmonary blastoma syndrome (a disorder that may cause cystic nephroma, lung cysts, thyroid problems, and cancers of the lung, kidney, ovary, and soft tissue).
DICER1 syndrome (a disorder that may cause a goiter, polyps in the colon, and tumors of the ovary, cervix, testicle, kidney, brain, eye, and lining of the lung).

Signs and symptoms of ovarian cancer include pain, swelling, or a lump in the abdomen.

These and other signs and symptoms may be caused by ovarian cancer or by other conditions.

Check with your child's doctor if your child has any of the following:

Pain or swelling in the abdomen.
A lump in the abdomen.
Constipation.
Painful or missed menstrual periods.
Unusual vaginal bleeding.
Male sex traits, such as body hair or a deep voice.
Early signs of puberty.

Tests that examine the ovaries are used to help diagnose ovarian cancer.

The following tests and procedures may be used:

Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the pelvis or abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
MRI (magnetic resonance imaging): A procedure that uses a magnet and radio waves to make a series of detailed pictures of areas inside the body, such as the pelvis or abdomen. The pictures are made by a computer. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child's abdomen helps make the pictures clearer.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs in the abdomen or pelvis and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
Biopsy: The tissue removed during surgery is viewed under a microscope by a pathologist to check for signs of cancer.
Serum tumor marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The tumor markers alpha-fetoprotein, beta-human chorionic gonadotropin (beta-hCG), CEA, CA-125, and others are used to diagnose ovarian cancer.

During surgery to remove the tumor, fluid in the abdomen will be checked for signs of cancer.

Stages of Childhood Ovarian Cancer

The process used to find out if cancer has spread from the ovary to nearby areas or to other parts of the body is called staging. There is no standard system for staging childhood ovarian cancer. The results of tests and procedures done to diagnose ovarian cancer are used to help make decisions about treatment.

Sometimes childhood ovarian cancer recurs (comes back) after treatment.

Treatment Option Overview

There are different types of treatment for children and adolescents with ovarian cancer.

Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children and adolescents with ovarian cancer should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

Pediatrician.
Pediatric surgeon.
Gynecologist.
Pediatric nurse specialist.
Rehabilitation specialist.
Social worker.
Psychologist.

Five types of standard treatment are used for ovarian cancer.

Surgery

Surgery is used to remove the cancer in the ovary. Surgery may also be used to remove the cancer and the ovary or the cancer and the ovary and fallopian tube.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can affect cancer cells throughout the body (systemic chemotherapy).

High-dose chemotherapy with autologous stem cell rescue

High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood -forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy and radiation therapy do.

Histone methyltransferase inhibitors: This type of targeted therapy slows down the cancer cell's ability to grow and divide. Tazemetostat is used to treat ovarian cancer.

Targeted therapy is also being studied for the treatment of childhood ovarian cancer that has recurred (come back).

Treatment for childhood ovarian cancer may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Physical problems, such as problems with fertility, are a late effect of treatment.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.