Rare Pulmonology News

Disease Profile

Adenosarcoma of the uterus

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)

Mullerian adenosarcoma of the uterus; Uterine adenosarcoma

Categories

Rare Cancers

Summary

Adenosarcoma of the uterus is a rare tumor of the uterus that typically originates in the lining of the uterus (endometrium).[1] This type of tumor is characterized by both benign (noncancerous) and malignant components (low-grade sarcoma).[1][2] Typical symptoms in affected individuals may include abnormal vaginal bleeding, an enlarged uterus, and tissue protruding from the external os (external opening of the uterus that leads into the cavity of the cervix).[1][3] It is most common in post-menopausal women but can affect women of any age.[3] Factors that may predisposed an individual to the condition include hyperestrogenemia (high blood estrogen levels), chemotherapy, or radiotherapy.[3] Recommended treatment includes hysterectomy, usually accompanied by bilateral salpingo-oophorectomy (removal of fallopian tubes and ovaries).[1] Ovarian conservation is an option for reproductive age women.[1]

Cause

The exact cause of adenosarcoma of the uterus is not always known. The development of these tumors has been associated with a few risk factors. Risk factors are associated with an increased risk of developing a condition; however, most people with these risk factors will never develop the condition. Factors that have reportedly been associated with development of adenosarcoma of the uterus include hyperestrinism (abnormally high levels of estrogenic hormones in the body), prior chemotherapy, prior radiotherapy and tamoxifen therapy (used as an adjuvant drug for breast cancer).[3][4][5]

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.

      Where to Start

      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • 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 Adenosarcoma of the uterus. Click on the link to view a sample search on this topic.

          References

          1. Xie YP, Yao HX, Shen YM. Müllerian adenosarcoma of the uterus with heterologous elements: two case reports and literature review. Arch Gynecol Obstet. August 2012; 286(2):537-540.
          2. Volkan Ulkera, Ekrem Yavuzb, Ali Gedikbasic, Ceyhun Numanoglua, Sinem Sudolmusc, Ahmet Gulkilika. Uterine adenosarcoma with ovarian sex cord-like differentiation: A case report and review of the literature. Taiwanese Journal of Obstetrics and Gynecology. December 2011; 50(4):518–521.
          3. Sinha A, Phukan JP, Sengupta S, Guha P. Mullerian adenosarcoma of uterus with sarcomatous overgrowth and heterologous component associated with stromal deposit in omentum: a case report and review of the literature. Case Report Med. 2012; ePub:
          4. Tjalma WA, Michener CM. Mullerian adenosarcoma of the uterus associated with long-term oral contraceptive use. Eur J Obstet Gynecol Reprod Biol. April 1, 2005; 119(2):253-254..
          5. Arici DS, Aker H, Yildiz E, Tasyurt A. Mullerian adenosarcoma of the uterus associated with tamoxifen therapy. Arch Gynecol Obstet. September 2000; 264(2):105-107.

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