[en] While most pituitary adenomas occur sporadically, nearly 5% of all cases may occur in a familial setting. Familial neoplasias have helped clinicians to elucidate molecular mechanisms of disease, in a cognitive process sequentially involving linkage analysis, mapping, and characterisation of pathogenically relevant genes. Most of familial cases are due to multiple endocrine neoplasia type 1 (MEN-1) and Carney's complex (CNC). In 2000, we described non-MEN1/CNC familial pituitary tumours that included all tumour phenotypes. This condition is named familial isolated pituitary adenomas (FIPA). We herein discusst some data of a retrospective and co-operative international study including Belgium, Italy, France and Germany. This study identified no consanguine families with two or more affected siblings, presenting with Growth hormone, Prolactin (1 pituitary cancer), gonadotroph (FSH, LH) , corticotroph (ACTH) and non functionning FIPA. This chapter describes the clinical features of familial pituitary adenomas like MEN1,CNC, and FIPA as well as the genetic/molecular data that have been recently reported in these syndromes.
