Rare Endocrinology News

Disease Profile

Adenoma of the adrenal gland

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)

Adrenal cortical adenoma; Adrenal adenoma; Adrenal incidentaloma;

Summary

Adenomas of the adrenal gland are non-cancerous (benign) tumors on the adrenal gland. Most do not cause any signs or symptoms and rarely require treatment. However, some may become "active" or "functioning" which means they produce hormones, often in excess of what the adrenal glands typically produce. High levels of these hormones can lead to complications, including primary aldosteronism, Cushing's syndrome and other medical conditions. Functioning adrenal adenomas may be treated with surgery and/or medications.[1][2]

Symptoms

The majority of adrenal adenomas are "nonfunctioning", which means they do not produce hormones and usually do not cause any symptoms. They are often found incidentally during imaging studies of the abdomen, in which case they are referred to as adrenal incidentalomas. However, some can become "functioning" or "active" and secrete excess hormones. Depending on the type of hormone released, adrenal tumors can cause a variety of conditions, including Cushing's syndrome, primary aldosteronism, or much less commonly, virilization.[3]

Cushing's syndrome, also called hypercortisolism (having abnormally high levels of cortisol), is caused by adrenal adenomas that release excess levels of the hormone cortisol. Common symptoms of Cushing's syndrome can include upper body obesity; severe fatigue and muscle weakness; high blood pressure; backache; high blood sugar; easy bruising; and bluish-red stretch marks on the skin. Affected women may have increased growth of facial and body hair, and menstrual periods may become irregular or stop completely.[4] Mild hypercortisolism without any obvious symptoms, called subclinical Cushing's syndrome, is common in people with an adrenal incidentaloma, although glucose intolerance and hypertension may be present in these cases.[3]

Primary aldosteronism (also called Conn syndrome) is a condition in which the adrenal gland produces too much of the hormone aldosterone. This hormone is responsible for balancing the levels of sodium and potassium in the blood. Symptoms of this condition may include high blood pressure, fatigue, headache, muscle weakness, numbness and paralysis that comes and goes.[5]

Benign cortisol-secreting adenomas can also produce small amounts of androgens (steroid hormones, such as testosterone), although androgen levels in the blood are usually not elevated.[3] Excess amounts of androgens can cause an increase in masculine characteristics (virilization) such as increased facial and body hair (hirsutism); deepening of the voice; increased muscularity; and other characteristics.[6]

Cause

The exact underlying cause of most adrenal adenomas is unknown. They sometimes occur in people with certain genetic syndromes such as multiple endocrine neoplasia, type 1 (MEN1) and familial adenomatous polyposis (FAP). In these cases, affected people usually have multiple adenomas and other characteristic features of the condition that are all caused by changes (mutations) in a gene. MEN1 is caused by mutations in the MEN1 gene, while FAP is caused by mutations in the APC gene.[7][8][9]

Diagnosis

In addition to a complete physical exam and medical history, the following imaging tests are usually necessary to diagnose an adenoma of the adrenal gland: computed tomography (CT scan), magnetic resonance imaging (MRI scan), and/or positron emission tomography (PET scan). Some people may also need a biopsy of the tumor to confirm the diagnosis. Laboratory tests that evaluate the levels of certain hormones in the blood or urine can be used to determine if the adrenal adenoma is functional or nonfunctional.[2]

Genetic testing is not available for many people with adrenal adenomas since most of these tumors occur sporadically (by chance) and are not caused by a genetic mutation.

However, genetic testing is an option for people with an inherited condition that predisposes to adrenal adenomas such as multiple endocrine neoplasia, type 1 (MEN1) and familial adenomatous polyposis (FAP).[9][8] Carrier testing for at-risk relatives and prenatal testing are possible if the disease-causing mutation in the family is known.

The Genetic Testing Registry (GTR) is a centralized online resource for information about genetic tests. It provides a list of laboratories performing genetic testing for MEN1 and FAP. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

Treatment

The best treatment options for adenomas of the adrenal gland depend on many factors, including whether or not the adenoma is "functional" (releasing hormones). People with adrenal adenomas that are not releasing hormones usually do not require any immediate treatment; however, they may need to follow up with a healthcare provider regularly to determine if the adenoma has grown and to make sure that it does not start producing hormones.[7]

Functional adrenal adenomas are typically treated with surgery. Removal of the affected adrenal gland usually resolves other medical conditions that may be present as a result of elevated adrenal hormones (i.e. primary aldosteronism, Cushing's syndrome). In some cases, functional adrenal adenomas can be treated with medications that block the function or lower the levels of the overproduced hormone.[4][6][5][7]

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

    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 Mayo Clinic has an information page titled "Benign Adrenal Tumors" on its Web site. Click on the link above to view this page.

      In-Depth Information

      • The Merck Manual for health care professionals provides information on Adenoma of the adrenal gland.
      • 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 Adenoma of the adrenal gland. Click on the link to view a sample search on this topic.

        References

        1. Benign Adrenal Tumors. Mayo Clinic. 2011; https://www.mayoclinic.org/benign-adrenal-tumor/. Accessed 10/24/2011.
        2. André Lacroix, MD. Clinical presentation and evaluation of adrenocortical tumors. UpToDate. Jul 28, 2014;
        3. Andre Lacroix. Adrenocortical Adenomas. UpToDate. Waltham, MA: UpToDate; 2011; https://www.uptodate.com/contents/clinical-presentation-and-evaluation-of-adrenocortical-tumors.
        4. NINDS Cushing's Syndrome Information Page. National Institute of Neurological Disorders and Stroke. July 2013; https://www.ninds.nih.gov/disorders/cushings/cushings.htm.
        5. Hyperaldosteronism Primary and Secondary. MedlinePlus. August 2013; https://www.nlm.nih.gov/medlineplus/ency/article/000330.htm.
        6. Ashley B. Grossman, MD, FRCP. Virilization. Merck Manual. September 2014; https://www.merckmanuals.com/home/hormonal_and_metabolic_disorders/adrenal_gland_disorders/virilization.html.
        7. George T Griffing, MD. Adrenal Incidentaloma. Medscape. September 2014; https://emedicine.medscape.com/article/116587-overview.
        8. Pagon RA, Adam MP, Ardinger HH, et al. Multiple Endocrine Neoplasia Type 1. GeneReviews. September 6, 2012; https://www.ncbi.nlm.nih.gov/books/NBK1538/?report=printable.
        9. Familial Adenomatous Polyposis. MedlinePlus Genetics. October 2013; https://medlineplus.gov/genetics/condition/familial-adenomatous-polyposis/.

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