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GTR Home > Conditions/Phenotypes > Somatotroph adenoma

Somatotroph adenoma

Synonyms
ACROMEGALY DUE TO PITUITARY ADENOMA 1; ISOLATED FAMILIAL SOMATOTROPINOMA; PITUITARY ADENOMA 1, MULTIPLE TYPES; Pituitary adenoma, growth hormone-secreting; Pituitary tumor, growth hormone-secreting, somatic; SOMATOTROPHINOMA, FAMILIAL

Summary

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.
Full text of GeneReview (by section):
Summary
Diagnosis
Clinical Characteristics
Genetically Related (Allelic) Disorders
Differential Diagnosis
Management
Genetic Counseling
Resources
Molecular Genetics
References
Chapter Notes
Authors:
Márta Korbonits
Ajith V Kumar
view full author information

Available tests

40 tests are in the database for this condition.

Clinical tests (40 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: ARA9, FKBP16, FKBP37, PITA1, SMTPHN, XAP-2, XAP2, AIP
    Summary: aryl hydrocarbon receptor interacting protein

Clinical features

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Imported from Human Phenotype Ontology (HPO)

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