Fallopian tube cancer develops in the tubes that connect a woman's ovaries and uterus. It is very rare and accounts for only 1-2% of all gynecologic cancers.

Fallopian tube cancer occurs when normal cells in one or both tubes change and grow in an uncontrolled way, forming a mass called a tumor. Cancer can begin in any of the different cell types that make up the fallopian tubes. The most common type is called adenocarcinoma (a cancer of cells from glands). Leiomyosarcoma (a cancer of smooth muscle cells) and transitional cell carcinoma (a cancer of the cells lining the fallopian tubes) are more rare.

While some fallopian tube cancers actually begin in the tubes themselves, fallopian tube cancer is more often the result of cancer spreading from other parts of the body to the tubes. For example, the fallopian tubes are a common site of metastasis (spread) of cancers that started in the ovaries, uterus, endometrium, (the tissue lining the uterus) appendix, or colon.

Women with fallopian tube cancer may experience symptoms, although some affected women may have no symptoms at all. The signs of fallopian tube cancer are often non-specific, meaning that they can also be signs of other medical conditions that are not cancer. Signs and symptoms of fallopian tube cancer can include: irregular or heavy vaginal bleeding (especially after menopause); occasional abdominal or pelvic pain or feeling of pressure; vaginal discharge that may be clear, white, or tinged with blood; and a pelvic mass or lump.

Doctors use many tests to diagnose cancer of the fallopian tubes. Some of these tests may include: pelvic examination, transvaginal ultrasound, a blood test that measures the tumor marker CA-125, computed tomography (CT or CAT) scan, and magnetic resonance imaging (MRI).

Fallopian tube cancer can be best treated when detected early. If the cancer has spread to the walls of the tubes or outside of the tubes, then there is a lower chance that the disease can be treated successfully. The stage of the cancer determines the type of treatment needed. Most women will need surgery and some will go on to have chemotherapy and/or radiation therapy.

General Information About Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer

KEY POINTS

• Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer are diseases in which malignant (cancer) cells form in the tissue covering the ovary or lining the fallopian tube or peritoneum.
• Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer form in the same type of tissue and are treated the same way.
• Women who have a family history of ovarian cancer are at an increased risk of ovarian cancer.
• Some ovarian, fallopian tube, and primary peritoneal cancers are caused by inherited gene mutations (changes).
• Women with an increased risk of ovarian cancer may consider surgery to lessen the risk.
• Signs and symptoms of ovarian, fallopian tube, or peritoneal cancer include pain or swelling in the abdomen.
• Tests that examine the ovaries and pelvic area are used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.
• Certain factors affect treatment options and prognosis (chance of recovery).

Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer are diseases in which malignant (cancer) cells form in the tissue covering the ovary or lining the fallopian tube or peritoneum.

The ovaries are a pair of organs in the female reproductive system. They are 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. The ovaries make eggs and female hormones (chemicals that control the way certain cells or organs work).

The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary.

The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary.

Ovarian epithelial cancer is one type of cancer that affects the ovary.

• Ovarian Germ Cell Tumors
• Ovarian Low Malignant Potential Tumors
• Childhood Ovarian Cancer Treatment

The digestive organs within the abdominal cavity are held in place by the peritoneum, a broad serous membranous sac made up of squamous epithelial tissue surrounded by connective tissue. It is composed of two different regions: the parietal peritoneum, which lines the abdominal wall, and the visceral peritoneum, which envelopes the abdominal organs (image). The peritoneal cavity is the space bounded by the visceral and parietal peritoneal surfaces. A few milliliters of watery fluid act as a lubricant to minimize friction between the serosal surfaces of the peritoneum.

The visceral peritoneum includes multiple large folds that envelope various abdominal organs, holding them to the dorsal surface of the body wall. Within these folds are blood vessels, lymphatic vessels, and nerves that innervate the organs with which they are in contact, supplying their adjacent organs. The five major peritoneal folds are described in the Table below. Note that during fetal development, certain digestive structures, including the first portion of the small intestine (called the duodenum), the pancreas, and portions of the large intestine (the ascending and descending colon, and the rectum) remain completely or partially posterior to the peritoneum. Thus, the location of these organs is described as retroperitoneal .

DISORDERS OF THE...

Digestive System: Peritonitis

Inflammation of the peritoneum is called peritonitis. Chemical peritonitis can develop any time the wall of the alimentary canal is breached, allowing the contents of the lumen entry into the peritoneal cavity. For example, when an ulcer perforates the stomach wall, gastric juices spill into the peritoneal cavity. Hemorrhagic peritonitis occurs after a ruptured tubal pregnancy or traumatic injury to the liver or spleen fills the peritoneal cavity with blood. Even more severe peritonitis is associated with bacterial infections seen with appendicitis, colonic diverticulitis, and pelvic inflammatory disease (infection of uterine tubes, usually by sexually transmitted bacteria). Peritonitis is life threatening and often results in emergency surgery to correct the underlying problem and intensive antibiotic therapy. When your great grandparents and even your parents were young, the mortality from peritonitis was high. Aggressive surgery, improvements in anesthesia safety, the advance of critical care expertise, and antibiotics have greatly improved the mortality rate from this condition. Even so, the mortality rate still ranges from 30 to 40 percent.

The primary female reproductive organs, or gonads, are the two ovaries. Each ovary is a solid, ovoid structure about the size and shape of an almond, about 3.5 cm in length, 2 cm wide, and 1 cm thick. The ovaries are located in shallow depressions, called ovarian fossae, one on each side of the uterus, in the lateral walls of the pelvic cavity. They are held loosely in place by peritoneal ligaments.

The ovaries are covered on the outside by a layer of simple cuboidal epithelium called germinal (ovarian) epithelium. This is actually the visceral peritoneum that envelops the ovaries. Underneath this layer is a dense connective tissue capsule, the tunica albuginea. The substance of the ovaries is distinctly divided into an outer cortex and an inner medulla. The cortex appears more dense and granular due to the presence of numerous ovarian follicles in various stages of development. Each of the follicles contains an oocyte, a female germ cell. The medulla is a loose connective tissue with abundant blood vessels, lymphatic vessels, and nerve fibers.

The ovaries are the female gonads. Paired ovals, they are each about 2 to 3 cm in length, about the size of an almond. The ovaries are located within the pelvic cavity, and are supported by the mesovarium, an extension of the peritoneum that connects the ovaries to the broad ligament. Extending from the mesovarium itself is the suspensory ligament that contains the ovarian blood and lymph vessels. Finally, the ovary itself is attached to the uterus via the ovarian ligament.

The ovary comprises an outer covering of cuboidal epithelium called the ovarian surface epithelium that is superficial to a dense connective tissue covering called the tunica albuginea. Beneath the tunica albuginea is the cortex, or outer portion, of the organ. The cortex is composed of a tissue framework called the ovarian stroma that forms the bulk of the adult ovary. Oocytes develop within the outer layer of this stroma, each surrounded by supporting cells. This grouping of an oocyte and its supporting cells is called a follicle. The growth and development of ovarian follicles will be described shortly. Beneath the cortex lies the inner ovarian medulla, the site of blood vessels, lymph vessels, and the nerves of the ovary. You will learn more about the overall anatomy of the female reproductive system at the end of this section.

Treatment Option Overview

KEY POINTS

• There are different types of treatment for patients with ovarian epithelial cancer.
• Three kinds of standard treatment are used.
  • Surgery
  • Chemotherapy
  • Targeted therapy
• New types of treatment are being tested in clinical trials.
  • Radiation therapy
  • Immunotherapy
• Treatment for ovarian epithelial, fallopian tube, and primary peritoneal cancer may cause side effects.
• Patients may want to think about taking part in a clinical trial.
• Patients can enter clinical trials before, during, or after starting their cancer treatment.
• Follow-up tests may be needed.

There are different types of treatment for patients with ovarian epithelial cancer.

Different types of treatment are available for patients with ovarian epithelial cancer. Some treatments are standard, 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 treatment currently used as standard treatment, the new treatment may become the standard treatment. Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Three kinds of standard treatment are used.

Surgery

Most patients have surgery to remove as much of the tumor as possible. Different types of surgery may include:

• Hysterectomy: Surgery to remove the uterus and, sometimes, the cervix. When only the uterus is removed, it is called a partial hysterectomy. When both the uterus and the cervix are removed, it is called a total hysterectomy. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy.

• Unilateral salpingo-oophorectomy: A surgical procedure to remove one ovary and one fallopian tube.
• Bilateral salpingo-oophorectomy: A surgical procedure to remove both ovaries and both fallopian tubes.
• Omentectomy: A surgical procedure to remove the omentum (tissue in the peritoneum that contains blood vessels, nerves, lymph vessels, and lymph nodes).
• Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the lymph node tissue under a microscope to check for cancer cells.

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 reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).

A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity.

Treatment with more than one anticancer drug is called combination chemotherapy.

The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells.

Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy.

Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer.

Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer.

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. Some women receive a treatment called intraperitoneal radiation therapy, in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer.

Immunotherapy

Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy.

Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer.

Treatment for ovarian epithelial, fallopian tube, and primary peritoneal cancer may cause side effects.

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.

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 condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

This page lists cancer drugs approved by the Food and Drug Administration (FDA) for ovarian, fallopian tube, or primary peritoneal cancer. The list includes generic and brand names. This page also lists common drug combinations used in these cancer types. The individual drugs in the combinations are FDA-approved. However, drug combinations themselves usually are not approved, but are widely used.

There may be drugs used in ovarian, fallopian tube, or primary peritoneal cancer that are not listed here.

Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer

• Alkeran (Melphalan)
• Alymsys (Bevacizumab)
• Avastin (Bevacizumab)
• Bevacizumab
• Carboplatin
• Cisplatin
• Cyclophosphamide
• Doxorubicin Hydrochloride
• Doxil (Doxorubicin Hydrochloride Liposome)
• Doxorubicin Hydrochloride Liposome
• Elahere (Mirvetuximab soravtansine-gynx)
• Gemcitabine Hydrochloride
• Gemzar (Gemcitabine Hydrochloride)
• Hycamtin (Topotecan Hydrochloride)
• Infugem (Gemcitabine Hydrochloride)
• Lynparza (Olaparib)
• Melphalan
• Mirvetuximab soravtansine-gynx
• Mvasi (Bevacizumab)
• Niraparib Tosylate Monohydrate
• Olaparib
• Paclitaxel
• Paraplatin (Carboplatin)
• Rubraca (Rucaparib Camsylate)
• Rucaparib Camsylate
• Tepadina (Thiotepa)
• Thiotepa
• Topotecan Hydrochloride
• Zejula (Niraparib Tosylate Monohydrate)
• Zirabev (Bevacizumab)

Drug Combinations Used in Ovarian, Fallopian Tube, or Primary Peritoneal Cancer

• BEP
• CARBOPLATIN-TAXOL
• GEMCITABINE-CISPLATIN
• JEB
• PEB
• VAC
• VeIP

Treatment of early ovarian epithelial cancer or fallopian tube cancer may include the following:

• Hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Lymph nodes and other tissues in the pelvis and abdomen are removed and checked under a microscope for cancer cells. Chemotherapy may be given after surgery.
• Unilateral salpingo-oophorectomy may be done in certain women who wish to have children. Chemotherapy may be given after surgery.

Treatment of advanced ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following:

• Hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Lymph nodes and other tissues in the pelvis and abdomen are removed and checked under a microscope to look for cancer cells. Surgery is followed by one of the following:
  • Intravenous chemotherapy.
  • Intraperitoneal chemotherapy.
  • Chemotherapy and targeted therapy (bevacizumab).
  • Chemotherapy and targeted therapy with a poly (ADP-ribose) polymerase (PARP) inhibitor.
• Chemotherapy and targeted therapy followed by surgery (possibly followed by intraperitoneal chemotherapy).
• Chemotherapy alone for patients who cannot have surgery.
• Targeted therapy with a PARP inhibitor (olaparib, rucaparib, niraparib).
• A clinical trial of targeted therapy with a PARP inhibitor (veliparib).

Treatment of recurrent ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following:

• Chemotherapy using one or more anticancer drugs.
• Targeted therapy with a poly (ADP-ribose) polymerase (PARP) inhibitor (olaparib, rucaparib, or niraparib) with or without chemotherapy.
• Chemotherapy and/or targeted therapy (bevacizumab).
• A clinical trial of hyperthermic intraperitoneal chemotherapy (HIPEC) during surgery.
• A clinical trial of a new treatment.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Bevacizumab(Brand name: Avastin) - Manufactured by Genentech, Inc.
  FDA-approved indication: June 2018 approved in combination with carboplatin and paclitaxel, followed by Avastin as a single agent, to treat patients with stage III or IV epithelial ovarian, fallopian tube, or primary peritoneal cancer following initial surgical resection. December 2016 approved either in combination with carboplatin and paclitaxel or in combination with carboplatin and gemcitabine, followed by Avastin as a single agent, to treat patients with platinum-sensitive recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer.
• Olaparib(Brand name: Lynparza) - Manufactured by AstraZeneca Pharmaceuticals LP
  FDA-approved indication: December 2018, olaparib (Lynparza) received expanded approval for the maintenance treatment of adult patients with deleterious or suspected deleterious germline or somatic BRCA-mutated (gBRCAm or sBRCAm) advanced epithelial ovarian, fallopian tube or primary peritoneal cancer who are in complete or partial response to first-line platinum based chemotherapy. Select patients with gBRCAm advanced epithelial ovarian, fallopian tube or primary peritoneal cancer for therapy based on FDA-approved companion diagnostic for Lynparza. Original approval was in August 2017.
• Rucaparib(Brand name: Rubraca) - Manufactured by Clovis Oncology, Inc.
  FDA-approved indication: April 2018 approved for the maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in complete or partial response to platinum-based chemotherapy.
• Niraparib(Brand name: Zejula) - Manufactured by Tesaro, Inc.
  FDA-approved indication: Indicated for maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemotherapy.