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Disease Profile

Ovarian carcinosarcoma

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

Adult

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ICD-10

C56

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)

Malignant mixed mullerian tumor of the ovary; Malignant mixed Müllerian tumor of the ovary; MMMT of the ovary;

Categories

Female Reproductive Diseases; Rare Cancers

Summary

Ovarian carcinosarcoma, also known as a malignant mixed mullerian tumor (MMMT) of the ovary, is a rare, aggressive cancer of the ovary with characteristics of two types of cancer: carcinoma and sarcoma.[1] Because women with this cancer often have no symptoms, more than half of women are diagnosed at an advanced stage.[2] When present, symptoms may include pain in the abdomen or pelvic area, bloating or swelling of the abdomen, quickly feeling full when eating, or other digestive problems. The cause of ovarian carcinosarcoma is not yet understood.[2] Treatment usually consists of surgery to remove the tumor and chemotherapy.[1] The chance of recovery and long-term survival (prognosis) is poor, with a reported 5-year survival rate of about 28%.[2]

Treatment

Because ovarian carcinosarcoma is rare, there are no established treatment guidelines. Treatment decisions are based on the unique features of each individual's diagnosis.[1] The National Comprehensive Cancer Network (NCCN), a group of physicians and researchers who strive to improve cancer care, recommends that women with ovarian carcinosarcoma be treated similarly to women with ovarian carcinoma (also called epithelial ovarian cancer), which is the most common type of ovarian cancer.[4]

Currently, treatment for ovarian carcinosarcoma usually begins with surgery to remove as much of the cancer as possible. Chemotherapy may be used to destroy any cancer cells that could be in the body after surgery. Medications that contain platinum (such as cisplatin or carboplatin) seem to be the most effective chemotherapies for ovarian carcinosarcoma. Recent evidence suggests that another medication called ifosfamide may increase the effectiveness of treatment when used in combination with platinum-based medications.[1][4]

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

    Social Networking Websites

      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

      • 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.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Ovarian carcinosarcoma. Click on the link to view a sample search on this topic.

        References

        1. del Carmen MG, Birrer M, Schorge JO. Carcinosarcoma of the ovary: a review of the literature. Gynecologic Oncology. 2012; 125:271-277. https://www.ncbi.nlm.nih.gov/pubmed/22155675.
        2. Kim HJ, Lee HM, Kim MK, Lee YK, Lee IH, Lee KH, Kim H. Prognostic assessment of sarcomatous histologic subtypes of ovarian carcinosarcoma. Obstet Gynecol Sci. July, 2017; 60(4):350-356. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5547082/.
        3. Sonoda Y, Saigo PE, Federici MG, Boyd J. Carcinosarcoma of the ovary in a patient with a germline BRCA2 mutation: evidence for monoclonal origin. Gynecologic Oncology. 2000; 76:226-229. https://www.ncbi.nlm.nih.gov/pubmed/10637076. Accessed 3/27/2013.
        4. National Comprehensive Cancer Network. Ovarian Cancer. NCCN Guidelines for Patients. 2017; https://www.nccn.org/patients/guidelines/ovarian/index.html.

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