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Disease Profile

Carcinoid 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

Adult

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ICD-10

-

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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Categories

Rare Cancers

Summary

A carcinoid tumor is a type of neuroendocrine tumor that usually develops in the digestive (GI) tract (such as the stomach or intestines) or in the lungs. In some cases, a carcinoid tumor develops in another part of the body, such as the pancreas, testicle (in men), or ovary (in women). It is a slow-growing tumor that typically does not cause symptoms in the early stages, so a person may have the tumor for years before being diagnosed. In later stages, symptoms may vary depending on where the tumor is located.[1][2][3] Symptoms of a GI carcinoid tumor may only develop if the tumor has spread to the liver. The tumor may produce hormone-like substances that spread to the body and cause symptoms of carcinoid syndrome, such as flushing of the face and chest, diarrhea, and trouble breathing. People with a lung carcinoid tumor are less likely to have carcinoid syndrome, but may experience coughing, wheezing, or pneumonia.[2] The tumor may also cause various symptoms if it has spread to other parts of the body.

The cause of carcinoid tumors is unknown, but certain unavoidable risk factors may increase a person's chance of developing a carcinoid tumor.[2] In general, cancer develops when a cell randomly develops mutations in its DNA.[4] Surgery to remove the tumor is the main treatment and can typically cure the tumor if it has not spread to other parts of the body.[1] Other treatment options may include radiation therapy, chemotherapy, and targeted therapy.[2]

Cause

The cause of carcinoid tumors is currently unknown. There are some unavoidable risk factors that may increase a person's risk of developing the tumor. It is important to note that people with several risk factors may never develop cancer, while others with no risk factors may develop cancer. Possible risk factors for carcinoid tumors include:[2][5][6]

Treatment

The resources below provide information about treatment options for this condition. If you have questions about which treatment is right for you, talk to your healthcare professional.

Management Guidelines

  • The North American Neuroendocrine Tumor Society has published several consensus guidelines relating to the medical treatment and management of neuroendocrine tumors. Guidelines are developed pursuant to National Institute of Health (NIH) standards and serve as important references for practicing physicians.

    FDA-Approved Treatments

    The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

    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

        • The American Cancer Society provides detailed guides on lung and gastrointestinal carcinoid tumors. Click on the above links to access this information.
        • Cancer.Net, a resource from the American Society of Clinical Oncology, provides information about this condition.
        • The Carcinoid Cancer Foundation, Inc. has an information page on carcinoid tumors and related neuroendocrine tumors. Click on Carcinoid Cancer Foundation, Inc. to view the information page.
        • 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.
        • The Neuroendocrine Tumor Research Foundation provides information about Carcinoid tumor.

          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 Carcinoid tumor. Click on the link to view a sample search on this topic.

            References

            1. Carcinoid Tumors. MedlinePlus. May 24, 2016; https://medlineplus.gov/carcinoidtumors.html.
            2. Carcinoid Tumor. Cancer.Net, American Society of Clinical Oncology. August, 2016; https://www.cancer.net/cancer-types/carcinoid-tumor/introduction.
            3. Newly Diagnosed: The Basics. The Carcinoid Cancer Foundation. https://www.carcinoid.org/for-patients/diagnosis/newly-diagnosed-the-basics/. Accessed 2/13/2018.
            4. Carcinoid Tumors. Mayo Clinic. December 3, 2015; https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/symptoms-causes/syc-20351039.
            5. What Are the Risk Factors for Gastrointestinal Carcinoid Tumors?. American Cancer Society. February 26, 2015; https://www.cancer.org/cancer/gastrointestinal-carcinoid-tumor/causes-risks-prevention/risk-factors.html.
            6. What Are the Risk Factors for Lung Carcinoid Tumors?. American Cancer Society. February 5, 2015; https://www.cancer.org/cancer/lung-carcinoid-tumor/causes-risks-prevention/risk-factors.html.

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