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AIP-Related Familial Isolated Pituitary Adenomas

MedGen UID:
489979
Concept ID:
CN169290
Disease or Syndrome

Definition

Familial isolated pituitary adenoma (FIPA) is an inherited condition characterized by development of a noncancerous tumor in the pituitary gland (called a pituitary adenoma). The pituitary gland, which is found at the base of the brain, produces hormones that control many important body functions.\n\nTumors 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.\n\nAnother 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.\n\nLess 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).\n\nIn 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.\n\nFamilial 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. [from MedlinePlus Genetics]

Recent clinical studies

Etiology

Marques P, Caimari F, Hernández-Ramírez LC, Collier D, Iacovazzo D, Ronaldson A, Magid K, Lim CT, Stals K, Ellard S, Grossman AB, Korbonits M; FIPA Consortium.
J Clin Endocrinol Metab 2020 Jun 1;105(6) doi: 10.1210/clinem/dgaa040. PMID: 31996917Free PMC Article
Rostomyan L, Potorac I, Beckers P, Daly AF, Beckers A
Ann Endocrinol (Paris) 2017 Jun;78(2):123-130. Epub 2017 May 5 doi: 10.1016/j.ando.2017.04.012. PMID: 28483363

Diagnosis

Marques P, Caimari F, Hernández-Ramírez LC, Collier D, Iacovazzo D, Ronaldson A, Magid K, Lim CT, Stals K, Ellard S, Grossman AB, Korbonits M; FIPA Consortium.
J Clin Endocrinol Metab 2020 Jun 1;105(6) doi: 10.1210/clinem/dgaa040. PMID: 31996917Free PMC Article
Rostomyan L, Potorac I, Beckers P, Daly AF, Beckers A
Ann Endocrinol (Paris) 2017 Jun;78(2):123-130. Epub 2017 May 5 doi: 10.1016/j.ando.2017.04.012. PMID: 28483363

Prognosis

Marques P, Caimari F, Hernández-Ramírez LC, Collier D, Iacovazzo D, Ronaldson A, Magid K, Lim CT, Stals K, Ellard S, Grossman AB, Korbonits M; FIPA Consortium.
J Clin Endocrinol Metab 2020 Jun 1;105(6) doi: 10.1210/clinem/dgaa040. PMID: 31996917Free PMC Article

Clinical prediction guides

Marques P, Caimari F, Hernández-Ramírez LC, Collier D, Iacovazzo D, Ronaldson A, Magid K, Lim CT, Stals K, Ellard S, Grossman AB, Korbonits M; FIPA Consortium.
J Clin Endocrinol Metab 2020 Jun 1;105(6) doi: 10.1210/clinem/dgaa040. PMID: 31996917Free PMC Article

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