Rare Pediatrics News

Disease Profile

Malignant mixed Mullerian tumor

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)

Mixed tumor, Mullerian; Mixed Mullerian tumor; Carcinosarcoma;

Categories

Rare Cancers

Summary

A malignant mixed müllerian tumor (MMMT), also called a carcinosarcoma, is a type of cancer that contains two types of cancer cells carcinoma and sarcoma cells. These tumors usually develop in tissues of the female genital tract and are associated with a poor outcome. The majority of these tumors arise in the uterus, though they can also occur in the ovaries, fallopian tubes, and cervix. Very rarely, MMMTs can develop in the female peritoneum (lining of the abdominal wall).[1][2][3]

Cause

Presently, most mixed Mullerian tumors (MMMT) are thought to occur by chance without an association to a genetic predisposition. There have been rare reports of MMMT of the uterus developing in individuals with Lynch syndrome, an inherited condition in which individuals have a genetic predisposition to develop certain cancers including uterine cancer.[4] One article in the medical literature describes two cases of MMMT of the ovary in women with personal and family histories of breast cancer, and the authors state that these cases of MMMT of the ovary are suggestive of hereditary breast-ovarian cancer syndrome. As such, these authors state that genetic testing should be considered in women with MMMT of the ovary.[5] Overall, there is currently very little information about the cause of MMMT.

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

      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.

        Where to Start

        • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

          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.
          • PubMed is a searchable database of medical literature and lists journal articles that discuss Malignant mixed Mullerian tumor. Click on the link to view a sample search on this topic.

            References

            1. General Information About Uterine Sarcoma. National Cancer Institute. November 2010; https://www.cancer.gov/cancertopics/pdq/treatment/uterinesarcoma/Patient. Accessed 9/16/2011.
            2. Banik T, Halder D, Gupta N, Dey P.. Malignant mixed Müllerian tumor of the uterus: Diagnosis of a case by fine-needle aspiration cytology and review of literature. Diagnostic Cytopathology. 2011; https://www.ncbi.nlm.nih.gov/pubmed/21472874. Accessed 4/18/2011.
            3. Gard G, Shah JP, & Kumar S et al. . Ovarian and uterine carcinosarcomas: a comparative analysis of prognostic variables and survival outcomes. Journal of Gynecological Cancer. 2010; 20(5):888-894. https://www.ncbi.nlm.nih.gov/pubmed/20606539. Accessed 9/16/2011.
            4. Lu KH, Daniels M. Endometrial and ovarian cancer in women with Lynch syndrome: update in screening and prevention. Familial Cancer. 2013; 12(2):273-277. https://www.ncbi.nlm.nih.gov/pubmed/23765559. Accessed 7/23/2014.
            5. Lavie O, Longacre T, Segev Y, Husain A. Ovarian carcinosarcomas associated with prolonged use of tamoxifen: case reports. International Journal of Gynecological Cancer. 2009; 19(9):1521-1523. https://www.ncbi.nlm.nih.gov/pubmed/19955929. Accessed 4/8/2015.
            6. Kanthan, Senger. Uterine Carcinosarcomas (Malignant Mixed Müllerian Tumours): A Review with Special Emphasis on the Controversies in Management. Obstetrics and Gynecology International. 2011; 10:1155. https://www.hindawi.com/journals/ogi/2011/470795/. Accessed 12/10/2012.

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