Familial isolated pituitary adenoma
Familial isolated pituitary adenoma (FIPA) is an inherited condition characterized by the development of noncancerous tumors in the pituitary gland.
Overview
Familial isolated pituitary adenoma (FIPA) involves the growth of a noncancerous tumor (pituitary adenoma) in the pituitary gland at the base of the brain. These tumors may release excess hormones or be nonfunctioning.
Common tumor types include:
- Prolactinomas: These release prolactin. In women, they may cause menstrual changes or unexpected breast milk production. In men, they may cause erectile dysfunction or decreased libido. Large tumors can press on nerves, affecting vision.
- Somatotropinomas: These release growth hormone. In children, they can cause gigantism (increased height), while in adults, they cause acromegaly (overgrowth of hands, feet, and face).
Other less common types include somatolactotropinomas, nonfunctioning adenomas, adrenocorticotropic hormone-secreting tumors (Cushing disease), thyrotropinomas, and gonadotropinomas. FIPA tumors are often larger and occur at a younger age than sporadic (non-inherited) pituitary adenomas.
Frequency
FIPA is rare, accounting for approximately 2 percent of all pituitary adenomas. More than 200 families with the condition have been described in medical literature.
Causes
FIPA can be caused by mutations in the AIP gene. The AIP protein is thought to act as a tumor suppressor, helping to prevent uncontrolled cell growth. Mutations in this gene impair the protein's function, allowing pituitary cells to divide unchecked. AIP mutations account for 15 to 25 percent of FIPA cases; the genetic causes for the remaining cases are currently unknown.
Inheritance
FIPA is inherited in an autosomal dominant pattern. However, it exhibits incomplete penetrance, meaning only 20 to 30 percent of individuals with an AIP mutation actually develop a pituitary adenoma.
