Pituitary adenoma in men and other endocrine neoplasia syndromes - 11/10/14
Résumé |
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.
Aim of the study |
Analyze the frequency and characteristics of pituitary adenomas in classic and atypical MEN diagnosed between 1981 and 2013.
Results |
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.
Conclusion |
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.
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Vol 75 - N° 5-6
P. 444 - octobre 2014 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.