Rare Gastroenterology News

Disease Profile

Granulosa cell tumor of the ovary

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

GCT of the ovary; Granulosa theca cell tumor; GTCT;

Categories

Rare Cancers

Summary

Granulosa cell tumor of the ovary is a rare type of ovarian cancer that accounts for approximately 2% of all ovarian tumors. This type of tumor is known as a sex cord-stromal tumor and usually occurs in adults. Granulosa cell tumors of the ovary cause higher than normal levels of estrogen in a woman's body. The symptoms of this excess estrogen depend on the woman's menstrual status; the most common symptoms include early puberty for affected young girls, an increase in abdomen size or irregularities of menstrual cycles in premenopausal women, and abnormal uterine bleeding in postmenopausal women. The exact cause of granulosa cell tumors is unknown. Treatment consists of surgery to remove the tumor, and additional treatments may also be used depending on the extent of the tumor.[1]

Treatment

Surgery is the first step of treating a granulosa cell tumor of the ovary and aims to remove as much of the tumor as possible. Additional treatments - such as radiation therapy, chemotherapy, or hormone therapy may follow surgery depending on the severity and extent of the original tumor, or if the tumor regrows after surgery (a recurrence). These treatments may improve survival or increase the length of disease-free time before a recurrence. After treatments are complete, individuals should continue to have regular visits with their physicians to check for recurrences. These visits should include updating the medical history, a pelvic examination, and possibly blood testing to look for chemicals produced by cancer cells (tumor markers). If there are any suspicious findings during these visits, an imaging test such as a CT scan may be done.[1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Granulosa cell tumor of the ovary. Click on the link to view a sample search on this topic.

        References

        1. Starks DC. Granulosa-Theca Cell Tumors. Medscape Reference. January 15, 2015; https://emedicine.medscape.com/article/254489-overview.
        2. Jessica E. Stine, Stuart Pierce, John T. Soper,. A Comprehensive Review of Diagnostic and Treatment Options for Granulosa Cell Tumors of the Ovary. OBSTETRICAL AND GYNECOLOGICAL SURVEY. 2014; 69(1):https://www-ncbi-nlm-nih-gov.ezproxy.nihlibrary.nih.gov/pubmed/25102249.

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