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Ovarian Germ Cell Tumors

Posted on November 24, 2021
Medically reviewed by
Howard Goodman, M.D.
Article written by
Jennifer Shuman

Ovarian germ cell tumors (OGCTs) are a type of ovarian cancer that develops in egg cells of the ovary. OGCTs may be malignant (cancerous) or benign (not cancerous). It is estimated that over 200,000 people worldwide are diagnosed with ovarian cancer annually. Cancerous OGCTs make up less than 2 percent of ovarian cancers, and they are usually diagnosed when a person is in their teens or 20s.

In this article, we’ll explain what you should know about OGCTs, including the cancer’s causes and symptoms, as well as diagnosis, treatment, and outlook.

Causes of Ovarian Germ Cell Tumors

Little is known about what exactly causes these ovarian cancers, partly because malignant germ cell tumors are so rare. In general, cancer occurs when cells grow and divide uncontrollably. OGCTs can arise after genetic mutations occur in ovarian cells. There are several types of mutations that can increase the risk of developing cancer. These mutations may turn on the cell cycle of growth and division permanently, or they may turn off the natural “brakes” of the cell cycle, so that growth continues even when it shouldn’t. Genetic mutations associated with OGCTs can be inherited from your parents, or the mutations can occur randomly.

In the United States, the incidence (rate) of OGCTs is slightly higher among Asian, Hispanic, and non-Hispanic Black women compared to non-Hispanic White, American Indian, and Native Alaskan women. Worldwide, OGCTs occur at slightly higher rates in East Asian and Central American women. These racial and ethnic differences suggest that there may be an undiscovered genetic predisposition to OGCTs.

Signs and Symptoms of Ovarian Germ Cell Tumors

There are often no signs or symptoms in the early stages of OGCT development, which can make diagnosis difficult. When symptoms do appear, common signs of OGCTs can include:

  • A lump or swelling in the abdomen without weight gain elsewhere
  • Unexplained weight loss
  • Pelvic pain or discomfort
  • Abdominal pain
  • Vaginal bleeding after menopause

Diagnosis and Staging of Ovarian Germ Cell Tumors

Diagnosis of OGCTs usually begins with a thorough physical exam and record of your personal and family health histories. If your doctor suspects ovarian cancer, they will likely take a blood sample and examine your ovaries, ovarian tissue, and pelvic area. A blood test can help your doctor determine whether certain tumor markers are present in the blood. A pelvic exam and a Pap smear are used to look for signs of disease in the vagina, uterus, and cervix. A CT scan may be done to look at the inside of your body for signs of a tumor.

When your doctor diagnoses ovarian cancer, they also determine the cancer’s stage. A cancer’s stage describes the size of the tumor and whether it has spread. The stage gives important information for determining the prognosis (outlook) and appropriate treatment.

Staging of OGCTs typically requires surgery to sample (biopsy) the peritoneal fluid, which lubricates the surface of tissues in the lower abdomen and pelvic cavity. Your surgeon may also remove any visible tumors and biopsy the surfaces of nearby organs. Your surgeon may remove lymph nodes as part of your surgery. Oncologists will look at the tissue biopsy samples to determine the ratio of immature cells to mature cells, the amount of abnormal cells in the tumor, and other factors that help them determine the grade of cancer. The grade is a measure of how aggressive the tumor may act.

Types of Ovarian Germ Cell Tumors

Within OGCTs, there are a few major subtypes of cancerous tumors: dysgerminomas, endodermal sinus tumors, and teratomas. There are also several other subtypes, which are rare. The subclassification of OGCT helps determine the optimal treatment regimen for each case. In about 5 percent of OGCT diagnoses, there is a mixed population of at least two subtypes.

Dysgerminoma

Dysgerminoma is the most common type of OGCT. It is usually diagnosed at an early stage in women of reproductive age. In 80 percent to 90 percent of cases, dysgerminoma only affects one ovary. The second ovary and other unaffected reproductive organs may often be spared during surgery to preserve fertility.

Early stage identification and treatment of dysgerminoma has long-term survival rates of up to 100 percent. The recurrence rate of dysgerminoma, or the chance that a person will develop the cancer again, is 15 percent to 25 percent. Recurrence is treated and cured in about 90 percent of cases with surgery and a combination chemotherapy called BEP (bleomycin, etoposide, and cisplatin) or EP (etoposide and cisplatin).

Endodermal Sinus Tumors

Yolk sac tumors, also known as endodermal sinus tumors, are the second-most common cancerous OGCT. Endodermal sinus tumors are usually diagnosed in people younger than 20 years old. Endodermal sinus tumors are aggressive and may spread to nearby organs (metastasize).

Due to recent advancements in treatment options, a fertility-preserving operation with BEP chemotherapy is the recommended standard of care. The five-year survival rate of endodermal sinus tumors can reach up to 90 percent.

Teratomas

Teratomas, which are usually diagnosed before a person turns 20, make up 10 percent to 20 percent of ovarian cancers diagnosed in people younger than 20. Immature teratomas are cancerous, and they are much less common than the benign mature teratoma (dermoid cyst). Cancerous, immature teratomas represent about 1 percent of all teratomas.

Immature teratomas usually form on only one ovary, which spares the second ovary and allows for the potential for fertility-sparing surgery. The five-year survival rate of people with immature teratomas can reach up to 94 percent if caught in an early stage.

Treatment for Ovarian Germ Cell Tumors

Treatment options for OGCTs have improved dramatically over the last 30 years, and new treatments have been developed that help preserve fertility. Treatment of OGCTs may include a health care team of gynecologic oncologists, medical oncologists, fertility specialists, and psychologists.

The standard of care for young people diagnosed with an OGCT is conservative surgery followed by chemotherapy with BEP. The aim of conservative surgery is to remove only the affected ovary, leaving the unaffected ovary, fallopian tubes, and uterus intact.

More aggressive surgery, such as a total hysterectomy, may also remove parts of the reproductive system that are not associated with the cancerous cells. However, more aggressive surgery has not been proven to have an advantage in survival outcomes compared to conservative surgery.

Some treatment plans may involve radiation therapy. This is more commonly used for dysgerminoma, which is more sensitive to radiation treatment than other forms of ovarian cancer. You may also want to ask your health care team about participating in clinical trials, which may expose you to newer therapies.

Prognosis of Ovarian Germ Cell Tumors

Overall, disease-free survival rates for women diagnosed with OGCTs are high. Though recurrence of OGCTs does happen, recurrent cancer can usually be successfully treated with second-line chemotherapy. Factors that increase the risk of worse outcomes include being older or having advanced stage or high-grade cancer (cancer with more abnormal cells present in the tumor).

Diagnosis of OGCT can cause anxiety and depression. Especially in cases where fertility cannot be preserved to treat the tumor, women have reported experiencing poor self-esteem and feelings of sexual inadequacy.

To help protect fertility, the National Institutes of Health funded and helped establish the Oncofertility Consortium in 2007. The goal of the Oncofertility Consortium is to help preserve fertility in cancer treatments across the world. Many women with OGCTs who have undergone fertility-sparing treatment have gone on to have successful pregnancies and healthy children.

Talk With Others Who Understand

MyOvarianCancerTeam is the social network for people with ovarian cancer and their loved ones. On MyOvarianCancerTeam, members come together to ask questions, give advice, and share their stories with others who understand life with ovarian cancer.

Are you living with ovarian germ cell tumors? Share your experience in the comments, or start a conversation by posting on MyOvarianCancerTeam.

Howard Goodman, M.D. is certified by the American Board of Obstetrics and Gynecology and specializes in the surgical management of women with gynecologic cancer. Review provided by VeriMed Healthcare Network.. Learn more about him here.
Jennifer Shuman is a graduate student at Vanderbilt University pursuing her Ph.D. in pathology, microbiology, and immunology. Learn more about her here.

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