Approximatively 15–20% of general FIPA kindreds and about 50% of homogeneous FIPA families with acromegaly-gigantism are caused by mutations/deletions in the aryl hydrocarbon receptor interacting protein ( AIP) gene. The most frequent familial form of pituitary adenoma is familial isolated pituitary adenoma (FIPA) syndrome. Known genetic syndromes associated with the occurrence of somatotropinomas include multiple endocrine neoplasia (MEN) type 1 and MEN 4, Carney complex, McCune–Albright syndrome (MAS), and pheochromocytoma/paraganglioma and pituitary adenoma syndrome (3PAs). The molecular genetics of these aggressive forms of somatotropinomas is of interest for research and clinical practice. GH-secreting pituitary tumors are predominantly sporadic lesions that occur in adults, although rare pediatric or familial forms can occur and these can have aggressive characteristics. Acromegaly and gigantism result from chronic excessive production and secretion of growth hormone (GH), usually by a pituitary adenoma, and are considered very rare conditions.
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