Pituitary adenomas are usually sporadic, but can be observed in Multiple Endocrine Neoplasias type 1 (MEN1) or in the Carney complex syndrome, Multiple Endocrine Neoplasias type 4 (MEN4) and in some hereditary pheochromocytomas or paragangliomas syndromes.

Analyze the frequency and characteristics of pituitary adenomas in classic and atypical MEN diagnosed between 1981 and 2013.

Ten men were observed in our practice: 6 were classified as MEN1 and 4 as atypical MEN which can be either NEM4 or hereditary pheochromocytomas-paragangliomas syndromes. Pituitary adenomas were found in 8 of 10 cases (80%). Four were associated to MEN1 and 4 to atypical MEN. 6/8 were macroadenomas with a median tumor height=19.88mm (7–50) and invasiveness in 2 cases. Among observed pituitary adenomas 2 were prolactinomas, 2 were somatotroph adenomas, 3 were non-secreting, and 1 was a gonadotroph adenoma. A certain degree of aggressiveness was noted as only 2 cases were good responders to treatment and others are still evolving with Ki67=5% in one case.

In our small series, pituitary adenomas are common in MEN1 and atypical MEN syndromes. Macroadenomas and non functioning tumors are prevailing, but have a variable aggressiveness. These syndromes genetically determined should have a systematic genetic study for a rapid diagnosis and an early management of the affected patients and their families.