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Disease Profile

Small Intestinal Adenocarcinoma

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.


US Estimated

Europe Estimated

Age of onset





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


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.


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.


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


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.


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.


Not applicable


Other names (AKA)

Adenocarcinoma of small instestine; Adenocarcinoma of the small bowel; Adenocarcinoma of the small instestine;


Digestive Diseases


Small intestine adenocarcinoma is one type of small bowel cancer, usually occurring in the duodenum or jejunum, that begins in the gland cells. The small intestine is part of the body’s digestive system, which also includes the esophagus, stomach, and large intestine.[1] The exact cause is still unknown, but it may be due to DNA changes in the small intestine adenocarcinoma cells.[2] The symptoms of small bowel tumors are often vague. The most common symptoms are pain in the belly (which may be the first symptom), weight loss, weakness, fatigue and low red blood cell counts (anemia).[3] The treatment and prognosis for small intestine cancer depends on its stage (tumor size and site) and the presence of metastases. For early stage cancer, surgery may be all that is needed. For more advanced cancer chemotherapy or radiation therapy may be required.[4]

Please visit the National Cancer Institute website for additional information:
Small Intestine Cancer Treatment (PDQ®)–Patient Version


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

      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.


        1. Small Bowel Cancer Overview. Cancer.Net. 2015; https://www.cancer.net/cancer-types/small-bowel-cancer/overview.
        2. Do we know what causes small intestine adenocarcinoma?. American Cancer Society. 2014; https://www.cancer.org/cancer/smallintestinecancer/detailedguide/small-intestine-cancer-what-causes.
        3. Signs and symptoms of small intestine adenocarcinoma. American Cancer Society. 2014; https://www.cancer.org/cancer/smallintestinecancer/detailedguide/small-intestine-cancer-signs-symptoms.
        4. How is small intestine adenocarcinoma staged?. American Cancer Society. 2014; https://www.cancer.org/cancer/smallintestinecancer/detailedguide/small-intestine-cancer-staging.