Characteristics of Familial Isolated Pituitary Adenomas

Adrian F Daly; Jean-François Vanbellinghen; Albert Beckers

Disclosures

Expert Rev Endocrinol Metab. 2007;2(6):725-733. 

In This Article

Five-year View

The description of FIPA has been followed by a steady increase in the number of families identified. Continued accrual of a greater number of patients over the coming years will help to confirm and extend the clinical characterization of FIPA. Clinically relevant topics, such as the response to treatment in FIPA compared with sporadic pituitary adenomas, remain to be explored. Similarly, in those FIPA kindreds with AIP mutations, much remains to be confirmed, including the penetrance of disease among mutation carriers and the responsiveness of patients with AIP mutations and pituitary adenomas to somatostatin analogs, dopamine agonists and surgery. Given that FIPA is an infrequent condition, international collaborations remain the best way to advance clinical understanding in this field. Genetic causes for the 85% of FIPA families (including the 50% of IFS families) that are negative for AIP mutations will be an important focus for research in the coming years. At this time, it is not known if patients with FIPA (particularly those with AIP mutations) have other definable associated endocrine/nonendocrine pathologies that would expand the disease definition beyond that of the pituitary. Ongoing studies are investigating these possibilities and data from experimental models (in vitro cell culture and knockout mice) may help to clarify this issue.

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