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Ancestor membership does not transfer phenotype values between groups.","importantHtml":""},"sections":[{"bodyHtml":"<p>FIPA can be caused by mutations in the AIP gene. The function of the protein produced from this gene is not well understood, but it is thought to act as a tumor suppressor, which means it helps prevent cells from growing and dividing in an uncontrolled way. Mutations in the AIP gene alter the protein or reduce the production of functional protein. These changes likely impair the ability of the AIP protein to control the growth and division of cells, allowing pituitary cells to grow and divide unchecked and form a tumor. It is not known why the pituitary gland is specifically affected or why certain types of pituitary adenomas develop.</p><p>AIP gene mutations account for approximately 15 to 25 percent of cases of FIPA. Somatotropinomas are the most common type of tumor in these individuals. The tumors usually occur at a younger age, often in childhood, and are larger than FIPA tumors not caused by AIP gene mutations. The other genetic causes of FIPA are unknown.</p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/familial-isolated-pituitary-adenoma/#causes\">MedlinePlus Genetics: Causes</a></p>","key":"forms","title":"What causes Familial isolated pituitary adenoma?"},{"bodyHtml":"<p>Tumors that form in the pituitary gland can release excess levels of one or more hormones, although some tumors do not produce hormones (nonfunctioning pituitary adenomas). Those that do are typically distinguished by the particular hormones they produce. Prolactinomas are the most common tumors in FIPA. These tumors release prolactin, a hormone that stimulates breast milk production in females. Both women and men can develop prolactinomas, although they are more common in women. In women, these tumors may lead to changes in the menstrual cycle or difficulty becoming pregnant. Some affected women may produce breast milk, even though they are not pregnant or nursing. In men, prolactinomas may cause erectile dysfunction or decreased interest in sex. Rarely, affected men produce breast milk. Large prolactinomas can press on nearby tissues such as the nerves that carry information from the eyes to the brain (the optic nerves), causing problems with vision.</p><p>Another type of tumor called somatotropinoma is also common in FIPA. These tumors release growth hormone (also called somatotropin), which promotes growth of the body. Somatotropinomas in children or adolescents can lead to increased height (gigantism), because the long bones of their arms and legs are still growing. In adults, growth of the long bones has stopped, but the tumors can cause overgrowth of the hands, feet, and face (acromegaly) as well as other tissues.</p><p>Less common tumor types in FIPA include somatolactotropinomas, nonfunctioning pituitary adenomas, adrenocorticotropic hormone-secreting tumors (which cause a condition known as Cushing disease), thyrotropinomas, and gonadotropinomas. In a family with the condition, affected members can develop the same type of tumor (homogenous FIPA) or different types (heterogenous FIPA).</p><p>In FIPA, pituitary tumors usually occur at a younger age than sporadic pituitary adenomas, which are not inherited. In general, FIPA tumors are also larger than sporadic pituitary tumors. Often, people with FIPA have macroadenomas, which are tumors larger than 10 millimeters.</p><p>Familial pituitary adenomas can occur as one of many features in other inherited conditions such as multiple endocrine neoplasia type 1 and Carney complex; however, in FIPA, the pituitary adenomas are described as isolated because only the pituitary gland is affected.</p><p class=\"section-source\">Source: <a href=\"https://medlineplus.gov/genetics/condition/familial-isolated-pituitary-adenoma\">MedlinePlus Genetics</a></p><details class=\"deep-dive\"><summary>Cushing disease</summary><p>Cushing disease is caused by elevated levels of a hormone called cortisol, which leads to a wide variety of signs and symptoms. This condition usually occurs in adults between the ages of 20 and 50; however, children may also be affected. The first sign of this condition is usually weight gain around the trunk and in the face. Affected individuals may get stretch marks (striae) on their thighs and abdomen and bruise easily. Individuals with Cushing disease can develop a hump on their upper back caused by abnormal deposits of fat. People with this condition can have muscle weakness, severe tiredness, and progressively thin and brittle bones that are prone to fracture (osteoporosis). They also have a weakened immune system and are at an increased risk of infections. Cushing disease can cause mood disorders such as anxiety, irritability, and depression. This condition can also affect a person&#x27;s concentration and memory. People with Cushing disease have an increased chance of developing high blood pressure (hypertension) and diabetes. Women with Cushing disease may experience irregular menstruation and have excessive hair growth (hirsutism) on their face, abdomen, and legs. Men with Cushing disease may have erectile dysfunction. Children with Cushing disease typically experience slow growth.</p><h3>Causes</h3><p>Cortisol protects the body from stress, maintains blood glucose (also called blood sugar) levels, and turns off (suppresses) inflammation. Cortisol is produced by the adrenal glands, which are small glands located at the top of each kidney. Normally, the production of cortisol is triggered by the release of a hormone called adrenocorticotropic hormone (ACTH) from the pituitary gland, which is located at the base of the brain.</p><p>In people with Cushing disease, noncancerous (benign) pituitary gland tumors (also called adenomas) release too much ACTH. The extra ACTH causes the adrenal glands to release too much cortisol, which leads to the signs and symptoms of Cushing disease.</p><p>The cause of Cushing disease is often unknown. However, genetic changes in certain genes cause Cushing disease in up to 70 percent of affected individuals. Genetic changes that cause disease are called pathogenic variants. The pathogenic variants that cause Cushing disease are typically somatic, which means they are acquired during a person&#x27;s lifetime and are present only in certain cells. The genes that are associated with Cushing disease often play a role in regulating the activity of hormones.</p><p>In some people with Cushing disease, pituitary adenomas contain cells that have a pathogenic variant in the USP8 gene. Pathogenic variants in the USP8 gene are the most common genetic cause of Cushing disease.</p><p>The USP8 gene provides instructions for making an enzyme that helps regulate the breakdown (metabolism) of certain proteins, including proteins that are involved in important cell signaling pathways. The pathogenic variants in the USP8 gene that are associated with Cushing disease cause cells in the pituitary adenoma to produce a version of the protein that does not switch off properly. As a result, certain signaling proteins become overactive, causing cells within the adenoma to release too much ACTH.</p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/cushing-disease/#causes\">MedlinePlus Genetics: Causes</a></p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/cushing-disease\">MedlinePlus: Cushing disease</a></p></details>","key":"overview","title":"Condition overview"}],"sharedSources":[],"slug":"familial-isolated-pituitary-adenoma","sourceDocuments":[{"name":"Familial isolated pituitary adenoma","reviewed":"2013-08","sections":{"causes":{"fetchedAt":"2026-10-20T20:21:25.574945+00:00","label":"Causes","pageSha256":"e60dcc7b2f53d521f61e5a8ddea64d89f5d6684b34b36e44c5de0b7c9e16773d","sourceUrl":"https://medlineplus.gov/genetics/condition/familial-isolated-pituitary-adenoma/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/familial-isolated-pituitary-adenoma"},{"name":"Cushing disease","reviewed":"2026-03","sections":{"causes":{"fetchedAt":"2026-10-20T20:20:47.386527+00:00","label":"Causes","pageSha256":"10ba9c3f7eb31b75b8d137a328e316c73750ebfaaf21fad2c72302fc11303a9b","sourceUrl":"https://medlineplus.gov/genetics/condition/cushing-disease/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/cushing-disease"}],"sourceNote":"MedlinePlus descriptions and Causes; original HPO annotations.","takeawayHtml":"Cause explanations are from MedlinePlus Genetics. Feature values below retain their original sources and patient groups.","title":"Familial isolated pituitary adenoma"},"schemaVersion":1}