Résumé

P454

Pituitary adenoma in men1 and other endocrine neoplasia syndromes

Dr FA. YAKERa, Pr NS. FEDALAb, Pr F. CHENTLIb

a Department of Endocrinology and Metabolic diseases, Bab El Oued teaching Hospital, Algiers, Algeria, Alger ; b Department of Endocrinology and Metabolic diseases, Bab El Oued teaching Hospital,Algiers,Algeria, Alger

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: 10 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 %). 4 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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