Ovarian, Fallopian Tube and Primary Peritoneal Cancer

Ovarian cancer refers to cancer that starts in the ovaries, fallopian tubes or peritoneum. Here’s some more information about each of these parts of the reproductive system:

  • Ovaries: The ovaries are part of the female reproductive system. The female body typically has two ovaries, which are located on either side of the uterus. The ovaries contain a woman’s eggs and release the hormones progesterone and estrogen. After a woman goes through menopause, the ovaries stop releasing eggs and producing certain hormones.
  • Fallopian tubes: The fallopian tubes connect the ovaries to the uterus (also called the womb). Most women have two fallopian tubes, one on either side of the uterus. Each month when a woman ovulates, an ovary releases an egg, which then travels through the fallopian tube toward the uterus.
  • Peritoneum: The peritoneum is the tissue that covers the inside of the abdomen and most abdominal organs. It lines the ovaries, fallopian tubes, uterus, bladder and rectum. Peritoneal fluid also covers the surfaces of these tissues, which helps them move around and not stick together.

Cancers of the ovaries, fallopian tubes and peritoneum are closely related and usually treated the same way. Peritoneal cancer is quite rare, so “ovarian cancer” typically describes cancer that started in the ovaries or fallopian tubes.

There are different types of ovarian and fallopian tube cancer, including:

  • Epithelial carcinoma: This accounts for 85 to 90% of ovarian and fallopian tube cancers.
  • Germ cell malignancies: This is a less common type of ovarian cancer affecting the cells that produce eggs. This type of cancer is more common in females ages 10 to 29.
  • Sex cord stromal tumors: This is a rare form of ovarian cancer in the connective tissue cells.
  • Fallopian tube cancer: Experts once thought fallopian tube cancer was rare, but they now believe many cases of ovarian cancer actually started in the fallopian tube.
There is currently no screening test for ovarian, fallopian tube and primary peritoneal cancer.

Most women who are diagnosed with ovarian cancer don’t fall into the “high-risk” category. However, there are some factors that may increase your chances of ovarian cancer:

  • Family history (having a mother, sister, grandmother or aunt with breast or ovarian cancer)
  • The BRCA1 or BRCA2 gene
  • A genetic mutation associated with Lynch syndrome
  • A personal history of breast, colorectal or uterine cancer
  • Endometriosis
  • Never giving birth
  • Starting your period earlier than age 12
  • Starting menopause after age 51
  • Unexplained infertility
  • Taking estrogen only hormone replacement therapy (HRT) by itself for 10 years or more
  • Being age 50 or older
  • Obesity in early adulthood
  • Being of North American, North European or Ashkenazi Jewish heritage

Currently, there are no known ways to prevent ovarian cancer. However, the following factors are associated with a lower risk of the disease:

  • Giving birth
  • Breastfeeding
  • Taking birth control pills for three or more years
  • Getting your tubes tied
  • Having a hysterectomy
  • Having both ovaries removed

Not every risk-reducing measure is right for every woman, so talk to your doctor and genetic counselor about your unique risk factors.

Ovarian cancer symptoms are often nonspecific and may be caused by or mistaken for other conditions. Signs to watch for include:

  • Abdominal pain
  • Abnormal vaginal bleeding or discharge
  • Back pain
  • Bloating
  • Constipation
  • Difficulty eating
  • Feeling full quickly during a meal
  • Frequent or more urgent need to urinate
  • Irregular menstrual cycles
  • Pain during sexual intercourse
  • Pressure or pain in the pelvic area
  • Swelling in the abdomen or pelvis
  • Weight loss

These symptoms can be common in women without ovarian cancer, so it’s important to pay attention to any changes in your body and note when your symptoms began.

Tell your doctor immediately if you have abnormal vaginal bleeding. Otherwise, seek medical care if you experience any of the other symptoms for two weeks or more. They may be caused by less serious conditions, but it is best to see your doctor if you notice any changes in your body.

If your primary care provider or gynecologist thinks you may have ovarian cancer, they may refer you to a gynecologic oncologist. The following tests can be used to diagnose ovarian cancer:

  • Abdominal/pelvic exam: Your doctor will check your ovaries, uterus, vagina, bladder and rectum for abnormalities, such as a growth.
  • Blood test: Your doctor may do a blood test to measure CA-125. CA-125 can be elevated in response to inflammation or stress, and your gynecologic oncologist may use it, along with other diagnostics to monitor treatment response. However, other conditions, such as endometriosis, uterine fibroids and pelvic inflammatory disease, can also cause elevated CA-125.
  • Transvaginal ultrasound: During this procedure, an ultrasound probe is inserted into the vagina and creates a picture of the ovaries and surrounding tissues using sound waves.
  • Computed tomography (CT) scan: A CT scan takes X-rays of the body from different angles to create a three-dimensional image that shows tumors or abnormalities.
  • Positron emission tomography (PET) scan: During a PET scan, a small amount of a radioactive sugar substance is injected into the body. Cells that use the most energy, such as cancer cells, absorb more of this substance than other cells. Then, a scanner creates images of your organs and tissues.
  • Magnetic resonance imaging (MRI): This test uses magnetic fields instead of X-rays to create images of the inside of the body. An MRI can also help your doctor measure the size of the tumor.
  • Paracentesis: During this procedure, some peritoneal fluid is removed from the abdomen and checked under a microscope for signs of cancer.
  • Biopsy: During a biopsy, a small sample of tissue is removed from the affected area of the body. This tissue is examined under a microscope for signs of cancer. A biopsy is the only way to make an official ovarian cancer diagnosis.
  • Molecular testing: Your oncologist may recommend that your tumor be tested to identify its proteins, genes and other factors.

Staging is based on where your cancer started, if or where it has spread, and if or how it’s affecting other areas of the body. Here are the stages of ovarian cancer:

  • Stage I: The cancer is only located in the ovaries and fallopian tubes and hasn’t spread to distant sites in the body or nearby lymph nodes.
    • Stage IA: The cancer is only found in one ovary or fallopian tube. There is no cancer on the surface of the ovaries or fallopian tubes or in the abdomen.
    • Stage IB: The cancer is found in both ovaries or fallopian tubes, but not in the peritoneal fluid or on the surface of the ovaries or fallopian tubes. 
    • Stage IC: The cancer is in one or both ovaries or fallopian tubes and:
      • Stage IC1: The tissue around the tumor broke during surgery, which potentially allowed cancer cells to leak into the abdomen and pelvis. 
      • Stage IC2: The cancer is found on the outer surface of the ovaries or fallopian tubes or the tissue around the tumor broke before surgery, potentially spilling cancer cells into the abdomen and pelvis.
      • Stage IC3: Cancer cells are detected in the fluid in the abdomen and pelvis.
  • Stage II: The cancer has spread from the ovaries or fallopian tubes to other organs in the pelvis, like the uterus, bladder, rectum or sigmoid colon, or there is primary peritoneal cancer. It hasn’t spread to the nearby lymph nodes or distant areas of the body.
    • Stage IIA: The cancer has spread to the uterus and fallopian tubes and/or ovaries.
    • Stage IIB: The cancer has spread to other areas of the pelvis.
  • Stage III: Cancer is found in one or both ovaries or fallopian tubes or is a primary peritoneal cancer. The cancer has spread to the retroperitoneal lymph nodes, located in the back of the abdomen, or to the peritoneum outside the pelvis.
    • Stage IIIA1(i): The cancer has spread to the retroperitoneal lymph nodes, located in the back of the abdomen, or to the peritoneum outside the pelvis. The metastases are 10 millimeters (mm) or less.
    • Stage IIIA1(ii): The cancer has spread to the retroperitoneal lymph nodes, located in the back of the abdomen, or to the peritoneum outside the pelvis. The metastases are greater than 10 mm.
    • Stage IIIA2: The cancer has microscopically spread to the abdomen from the pelvis.
    • Stage IIIB: The cancer has visibly spread to the abdomen from the pelvis. The tumor is 2 centimeters (cm) or less. It may or may not have spread to the retroperitoneal lymph nodes.
    • Stage IIIC: The cancer has visibly spread to the abdomen from the pelvis and is larger than 2 cm. It may or may not have spread to the retroperitoneal lymph nodes.
  • Stage IV:
    • Stage IVA: The cancer has spread to the fluid surrounding the lungs.
    • Stage IVB: The cancer has spread to the spleen or liver, organs outside of the abdomen, or the lymph nodes in the groin.

In addition to staging the cancer, your doctor will also grade it to determine how much it looks like healthy cells under a microscope. Grades include:

  • Grade 1: The tumor tissue is well-differentiated and may grow slowly.
  • Grade 2: The tissue is moderately differentiated (this is a less common grade).
  • Grade 3: The tissue is poorly differentiated or undifferentiated, meaning that most or all cells in the tumor look abnormal. Grade 3 tumors often grow and spread quickly.

Your doctor will use staging and grading to determine what type of treatment is right for you.

During ovarian cancer treatment, you’ll be cared for by a multidisciplinary team. This team may include:

  • A gynecologic oncologist
  • A medical
  • A radiation oncologist
  • A nurse navigator
  • Nurse practitioners
  • Physician assistants
  • Oncology nurses
  • Social workers
  • Dietitians
  • Counselors
  • Pharmacists

Once ovarian cancer is diagnosed, one or more treatment options may be recommended. The treatment will depend on the type and stage of the disease and may include:

  • Surgery: The extent and type of surgery is based on your general health and the stage and type of the cancer.
    • For epithelial ovarian cancer, the primary goals of surgery are staging (determining if and how much the cancer has spread) and debulking (removing as much of the tumor as possible). Surgical options can include:
      • Partial hysterectomy (removing only the uterus)
      • Total hysterectomy (removing the uterus and the cervix)
      • Unilateral salpingo-oophorectomy (removing only one ovary and one fallopian tube)
      • Bilateral salpingo-oophorectomy (removing both ovaries and both fallopian tubes)
    • For germ cell tumors and stromal tumors, surgery (including hysterectomy and unilateral or bilateral salpingo-oophorectomy) is done to remove the cancer, with additional debulking surgery if necessary.
    • Other surgical procedures that may be included in the treatment plan include:
      • Omentectomy (removing the omentum, the tissue in the peritoneum that contains blood vessels, nerves, lymph vessels and lymph nodes)
      • Lymph node dissection or lymphadenectomy (removing all or part of a lymph node to check for cancer cells)
  • Radiation therapy: Radiation therapy for patients with ovarian cancer is used primarily to treat areas where the cancer has spread, rather than as a form of treatment for the primary tumor. It can be administered either as external radiation therapy (from outside the body) or internal radiation therapy (a radioactive substance is placed in or near the tumor).
  • Chemotherapy: Chemotherapy uses specific drugs to slow or kill cancer cells. With systemic chemotherapy, the medication is injected into a vein or given orally for specific types of ovarian cancer. The goal of chemotherapy in early-stage ovarian cancer is to decrease the chance of the cancer coming back. It can also be injected through a catheter directly into the abdominal cavity, called intraperitoneal chemotherapy or regional chemotherapy.
  • Targeted therapy: Targeted therapy is designed to identify and attack specific cancer cells without harming normal cells. Targeted therapies can also be used to activate cells to carry drugs, toxins or radioactive material directly to cancer cells.
  • Hormone therapy: Hormone therapy uses hormones or hormone-blocking drugs to treat ovarian stromal tumors. Hormone therapy can include:
    • Luteinizing-hormone-releasing hormone (LHRH) agonists that stop estrogen production by the ovaries
    • Tamoxifen that stops estrogen from stimulating cancer cell growth
    • Aromatase inhibitors that block the aromatase enzyme from converting hormones into estrogen in post-menopausal women

New treatment options continue to be studied in clinical trials, such as immunotherapy, which uses the patient’s immune system to fight cancer by boosting, directing or restoring the body's natural defenses against cancer.

Robotic Surgery for Ovarian Cancer Treatment

Your gynecologic oncologist may recommend robotic surgery, a type of minimally invasive surgery. Robotic surgery gives your doctor more control, a better view and high-definition 3D vision during the procedure. Because this type of surgery requires just a few small incisions, you may experience:

  • Quicker recovery
  • Less blood loss and transfusions
  • Reduced pain
  • Less scarring
  • Lower risk of infection

Ask your doctor if you’re eligible for robotic surgery.

If you have a personal or family history of breast or ovarian cancer, ask your physician if you should consider genetic counseling. Genetic counseling helps people determine their risk for hereditary cancers. Genetic counselors are medical specialists who help people:

  • Identify their risk of hereditary forms of cancer
  • Develop a personalized plan to manage risk

What Happens During Genetic Counseling?

First, you will provide your family medical history. From the history, your genetic counselor determines the likelihood that cancer in your family is hereditary. Your genetic counselor will discuss with you the following:

  • Your need for genetic testing
  • Any inheritance patterns in your family
  • Your surveillance and risk reduction options

If you choose to do testing, your genetic counselor will coordinate the testing. After testing, your genetic counselor will interpret the results to come up with the best surveillance and risk reduction plan for you. Your genetic counselor will also tell you if other members of your family need to be tested.

What Are the Most Common Hereditary Cancer Syndromes?

Genetic testing for hereditary breast and ovarian cancer and Lynch syndrome are the most common reasons for referrals to genetic counselors.

What are BRCA Mutations?

The BRCA1 and BRCA2 gene mutations make up the majority of hereditary breast and ovarian cancers.

Women who carry mutations in the BRCA genes have a 55 to 85% risk of developing breast cancer and a 15 to 60% risk of developing ovarian cancer. For women who do not carry mutations in the BRCA genes, the risk is 12 to 13% for breast cancer and 1 to 2% for ovarian cancer.

Men who carry mutations in the BRCA genes have a slightly increased risk of prostate cancer. Men who carry BRCA2 mutations have an increased risk of male breast cancer, pancreatic cancer and perhaps melanoma.

Should I Do Genetic Testing?

If one or more of these apply to you or your family, talk to your healthcare team about genetic testing:

  • Multiple family members on the same side of the family with the same cancer
  • Multiple family members on the same side of the family with related cancers (breast/ovarian/pancreatic or colon/uterine/ovarian)
  • Cancer at early ages (under 50)
  • More than one diagnosis of cancer in the same person
  • Rare cancers
  • A known family history of altered genes that increase cancer risk
  • Triple-negative breast cancer diagnosis before 60
  • Jewish ancestry
  • A personal or family history of breast, ovarian or pancreatic cancer
  • A pheochromocytoma or a paraganglioma tumor

If you are concerned about hereditary cancers, speak to your healthcare team or find a genetic counselor.

Where Can I Find a Genetic Counselor?

You can find a genetic counselor through the National Society of Genetic Counselors. You can also ask your healthcare team for a referral.

Will My Insurance Cover Genetic Testing?

Most insurance companies now cover genetic testing for people with a family history that suggests a hereditary cancer syndrome. Your genetic counselor can help you verify your coverage.

If you are uninsured or genetic testing is not covered by your insurance, your genetic counselor can help you locate local and national resources.

I’ve Decided to Meet with a Genetic Counselor. How Should I Prepare?

You need to give your genetic counselor as much accurate and detailed information as possible. Your genetic counselor will want to know about:

  • Your personal and/or family history of cancer
  • Who was diagnosed? Which side of the family?
  • What was the diagnosis?
  • What was their age at diagnosis?
  • Pathology reports
  • Death certificates
  • Age and cause of death of all family members
  • Your ethnic background
  • Any Jewish ancestry

Even if you have little or no information about your family history, you can still speak with a genetic counselor.

I Tested Positive for a Hereditary Cancer Syndrome. Now What Do I Do?

Your genetic counselor will help you come up with the best surveillance and risk reduction plan based on your risk. You may be referred to a doctor who can advise you about how to prevent the cancer identified, sometimes through surgery to remove the organ that could develop cancer. Be sure to always follow your recommended screening schedule. Cancer is easier to treat when caught early.

Your genetic counselor can help you share your test results and coordinate testing for family members. Your family needs to know about surveillance and risk reduction options. Some family members may not carry the same mutation.

If you have ovarian cancer, you may be wondering if you’ll still be able to have children after treatment. If you’re pre-menopausal, talk with your physician about possible ways to preserve your fertility. You may be referred to a fertility specialist called a reproductive endocrinologist before you begin cancer treatment. Be sure to ask which fertility treatments (if any) your insurance will cover.

See Sarah Cannon’s blog on fertility preservation for more information.