Familial pituitary adenomas

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Abstract

<jats:title>Abstract.</jats:title><jats:p>The majority of pituitary adenomas occur sporadically, however, about 5% of all cases occur in a familial setting, of which over half are due to multiple endocrine neoplasia type 1 (MEN‐1) and Carney’s complex (CNC). Since the late 1990s we have described non‐MEN1/CNC familial pituitary tumours that include all tumour phenotypes, a condition named familial isolated pituitary adenomas (FIPA). The clinical characteristics of FIPA vary from those of sporadic pituitary adenomas, as patients with FIPA have a younger age at diagnosis and larger tumours. About 15% of FIPA patients have mutations in the <jats:italic>aryl hydrocarbon receptor interacting protein</jats:italic> gene (<jats:italic>AIP</jats:italic>), which indicates that FIPA may have a diverse genetic pathophysiology. This review describes the clinical features of familial pituitary adenomas like MEN1, the MEN 1‐like syndrome MEN‐4, CNC, FIPA, the tumour pathologies found in this setting and the genetic/molecular data that have been recently reported.</jats:p>

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