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Prolactin-producing pituitary gland adenoma

MedGen UID:
10936
Concept ID:
C0033375
Neoplastic Process
Synonyms: Pituitary prolactin cell adenoma; Prolactinoma, familial
SNOMED CT: Prolactin-secreting pituitary adenoma (134209002); Prolactinoma (134209002); Prolactinoma (34337008)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
HPO: HP:0006767
Monarch Initiative: MONDO:0010911
Orphanet: ORPHA2965

Disease characteristics

Excerpted from the GeneReview: AIP Familial Isolated Pituitary Adenomas
AIP familial isolated pituitary adenoma (AIP-FIPA) is defined as the presence of an AIP germline pathogenic variant in an individual with a pituitary adenoma (regardless of family history). The most commonly occurring pituitary adenomas in this disorder are growth hormone-secreting adenomas (somatotropinoma), followed by prolactin-secreting adenomas (prolactinoma), growth hormone and prolactin co-secreting adenomas (somatomammotropinoma), and nonfunctioning pituitary adenomas (NFPA). Rarely TSH-secreting adenomas (thyrotropinomas) are observed. Clinical findings result from excess hormone secretion, lack of hormone secretion, and/or mass effects (e.g., headaches, visual field loss). Within the same family, pituitary adenomas can be of the same or different type. Age of onset in AIP-FIPA is usually in the second or third decade. [from GeneReviews]
Authors:
Márta Korbonits  |  Ajith V Kumar   view full author information

Additional description

From OMIM
Prolactin-secreting pituitary adenoma, or prolactinoma, is the most common type of hormonally active pituitary adenoma. These tumors can also be seen as a feature of multiple endocrine neoplasia type I (MEN1; 131100). See also 102200 for a discussion of familial isolated pituitary adenoma (FIPA) and acromegaly due to a growth hormone (GH; 139250)-secreting pituitary adenoma, which are also caused by mutation in the AIP gene. Schlechte (2003) discussed prolactinoma in general terms as a clinical, diagnostic, and therapeutic problem.  http://www.omim.org/entry/600634

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProlactin-producing pituitary gland adenoma
Follow this link to review classifications for Prolactin-producing pituitary gland adenoma in Orphanet.

Conditions with this feature

Multiple endocrine neoplasia, type 1
MedGen UID:
9957
Concept ID:
C0025267
Neoplastic Process
Multiple endocrine neoplasia type 1 (MEN1) includes varying combinations of more than 20 endocrine and non-endocrine tumors. Endocrine tumors become evident either by overproduction of hormones by the tumor or by growth of the tumor itself. Parathyroid tumors are the most common MEN1-associated endocrinopathy; onset in 90% of individuals is between ages 20 and 25 years with hypercalcemia evident by age 50 years; hypercalcemia causes lethargy, depression, confusion, anorexia, constipation, nausea, vomiting, diuresis, dehydration, hypercalciuria, kidney stones, increased bone resorption/fracture risk, hypertension, and shortened QT interval. Pituitary tumors include prolactinoma (the most common), which manifests as oligomenorrhea/amenorrhea and galactorrhea in females and sexual dysfunction in males. Well-differentiated endocrine tumors of the gastro-entero-pancreatic (GEP) tract can manifest as Zollinger-Ellison syndrome (gastrinoma); hypoglycemia (insulinoma); hyperglycemia, anorexia, glossitis, anemia, diarrhea, venous thrombosis, and skin rash (glucagonoma); and watery diarrhea, hypokalemia, and achlorhydria syndrome (vasoactive intestinal peptide [VIP]-secreting tumor). Carcinoid tumors are non-hormone-secreting and can manifest as a large mass after age 50 years. Adrenocortical tumors can be associated with primary hypercortisolism or hyperaldosteronism. Non-endocrine tumors include facial angiofibromas, collagenomas, lipomas, meningiomas, ependymomas, and leiomyomas.
Somatotroph adenoma
MedGen UID:
1618709
Concept ID:
C4538355
Neoplastic Process
AIP familial isolated pituitary adenoma (AIP-FIPA) is defined as the presence of an AIP germline pathogenic variant in an individual with a pituitary adenoma (regardless of family history). The most commonly occurring pituitary adenomas in this disorder are growth hormone-secreting adenomas (somatotropinoma), followed by prolactin-secreting adenomas (prolactinoma), growth hormone and prolactin co-secreting adenomas (somatomammotropinoma), and nonfunctioning pituitary adenomas (NFPA). Rarely TSH-secreting adenomas (thyrotropinomas) are observed. Clinical findings result from excess hormone secretion, lack of hormone secretion, and/or mass effects (e.g., headaches, visual field loss). Within the same family, pituitary adenomas can be of the same or different type. Age of onset in AIP-FIPA is usually in the second or third decade.

Professional guidelines

PubMed

Inder WJ, Jang C
Medicina (Kaunas) 2022 Aug 13;58(8) doi: 10.3390/medicina58081095. PMID: 36013562Free PMC Article
Molitch ME
JAMA 2017 Feb 7;317(5):516-524. doi: 10.1001/jama.2016.19699. PMID: 28170483
Melmed S, Casanueva FF, Hoffman AR, Kleinberg DL, Montori VM, Schlechte JA, Wass JA; Endocrine Society
J Clin Endocrinol Metab 2011 Feb;96(2):273-88. doi: 10.1210/jc.2010-1692. PMID: 21296991

Recent clinical studies

Diagnosis

Mittelbronn M, Psaras T, Capper D, Meyermann R, Honegger J
Neuro Endocrinol Lett 2006 Feb-Apr;27(1-2):89-92. PMID: 16648816

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