Ovarian cancer

What causes ovarian cancer?

Most cases of ovarian cancer occur sporadically in people with little to no family history of the condition. They are due to random changes (mutations) that occur only in the cells of the ovary. These mutations (called somatic mutations) accumulate during a person's lifetime and are not inherited or passed on to future generations.

However, approximately 10-25% of ovarian cancers are thought to be "hereditary." These cases are caused by an inherited predisposition to ovarian cancer that is passed down through a family. In some of these families, the underlying genetic cause is not known. However, many of these cases are part of a hereditary cancer syndrome. For example, the following cancer syndromes are associated with an elevated risk of ovarian cancer and several other types of cancer:

Of note, some research suggests that inherited mutations in several other genes (including BARD1 ,BRIP1, MRE11A, NBN, RAD51, RAD50, CHEK2, _and PALB2_) may also be associated with an increased risk for ovarian cancer. However, the risk associated with many of these genes is not well understood. Most are termed "moderate- or low- penetrant" genes which means that, on their own, they would be expected to have a relatively small effect on ovarian cancer risk. However, in combination with other genes and/or environmental factors, these genes may lead to a significant risk of ovarian cancer.

Last updated on 05-01-20

How is ovarian cancer diagnosed?

If ovarian cancer is suspected based on the presence of certain signs and symptoms, the following tests and procedures may be recommended:

  • Physical evaluation and pelvic exam to check for signs of ovarian cancer (i.e. lumps or swelling) and to examine the size, shape and location of the ovaries
  • Ultrasound and other imaging studies to look for abnormal growths
  • Blood tests such as a CA-125 assay which may be elevated in people with ovarian cancer
  • A biopsy of the tumor is the only way to confirm a diagnosis of ovarian cancer

The National Cancer Institute offers more detailed information regarding the tests used to diagnose ovarian cancer. To access this resource, please click here.

Last updated on 05-01-20

Is ovarian cancer inherited?

Most cases of ovarian cancer occur sporadically in people with little to no family history of the condition. However, approximately 10-25% of ovarian cancer is thought to be inherited. In some of these families, the underlying genetic cause is not known. However, many of these cases are part of a hereditary cancer syndrome such as BRCA1 or BRCA2 hereditary breast and ovarian cancer syndrome, Lynch syndrome and Peutz-Jeghers syndrome, which are inherited in an autosomal dominant manner. This means that a person only needs a change (mutation) in one copy of the responsible gene in each cell to have a hereditary predisposition to ovarian cancer and other cancers associated with these syndromes. In some cases, an affected person inherits the mutation from an affected parent. Other cases may result from new (de novo) mutations in the gene. A person with one of these syndromes has a 50% chance with each pregnancy of passing along the altered gene to his or her child.

Last updated on 05-01-20

What is the long-term outlook for people with ovarian cancer?

The long-term outlook (prognosis) for women with ovarian cancer depends on many factors including the subtype of cancer and the stage at which the cancer is diagnosed. In general, epithelial ovarian cancers (the most common subtype) are often associated with a worse prognosis than more rare subtypes such as germ cell and stromal ovarian tumors.

Because early stages of ovarian cancer are often not associated with any specific signs or symptoms, many cases are, unfortunately, diagnosed at a later and less treatable stage. Late stage ovarian cancers are generally associated with a poor prognosis. For example, 5-year survival rates for women diagnosed with stage I ovarian cancer range from 79-87%, while the 5-year survival rates for women diagnosed with stage IV ovarian cancer are approximately 11%.

Last updated on 05-01-20

How might ovarian cancer be treated?

The best treatment options for ovarian cancer depend on many factors including the subtype and stage of the condition. In general, treatment may include a combination of the following:

Unfortunately, research suggests that there are no benefits of ovarian cancer screening (using a CA-125 blood test and transvaginal ultrasound), even in women who are at an elevated risk due to their personal and/or family histories. Women who are at a high-risk for ovarian cancer may, therefore, want to discuss other preventative measures, such as prophylactic surgery, with their healthcare provider. In women with a known hereditary cancer syndrome, management should also include screening for the other component cancers. Please click on the following links for more information regarding the treatment and management of each condition:

Last updated on 05-01-20

Name: American Cancer Society 250 Williams Street NW
Atlanta, GA, 30329, United States
Toll Free: 1-800-227-2345 Url: https://www.cancer.org
Name: Ovarian Cancer Research Alliance 14 Pennsylvania Plaza, Suite 2110
New York, NY, 10122, United States
Phone: 212-268-1002 Toll Free: 866-399-6262 Fax : 202-331-2292 Email: info@ocrahope.org Url: https://ocrahope.org
Name: National Ovarian Cancer Coalition 2501 Oak Lawn Avenue, Suite 435
Dallas, TX, 75219 , United States
Phone: 214-273-4200 Toll Free: 888-OVARIAN Fax : 214-273-4201 Email: nocc@ovarian.org Url: http://www.ovarian.org/
Name: Minnesota Ovarian Cancer Alliance 4604 Chicago Ave
Minneapolis, MN, 55407,
Phone: 612-822-0500 Email: INFO@MNOVARIAN.ORG Url: http://mnovarian.org/
Ana Rath and Stéphanie NGUENGANG WAKAP. Prevalence of rare diseases: Bibliographic data », Orphanet Report Series, Rare Diseases collection, January 2020, Number 2 : Diseases listed by decreasing prevalence, incidence or number of published cases Orphanet Report Series - Prevalence of rare diseases: Bibliographic data - January 2020 - Number 2. January 2020; Number 2. 64. Reference Link

Note, these links are external searches against the National Laboratory of Medicine's drug database. You may need to adjust the search if there are no results found.

Drug Name Generic Name
Zejula niraparib
Lynparza olaparib
Infugem gemcitabine ready-to-use
Avastin bevacizumab
Rubraca rucaparib
Doxil Doxorubicin liposome
Ethyol Amifostine
Oncoscint Cr/Ov Satumomab pendetide
Hexalen Altretamine

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