Pituitary Adenomas in Multiple Endocrine Neoplasias Type1 and Other Endocrine Neoplasias Syndromes (Pages 42-46)

 

Fetta-Amel Yaker and Farida Chentli
Department of Endocrinology and Metabolic Diseases, Bab El Oued Teaching Hospital. 5, Boulevard Said Touati, Algiers, Algeria
 
DOI: http://dx.doi.org/10.12970/2310-9971.2015.03.02.3

Abstract: Multiple Endocrine Neoplasias (MEN) are very rare genetic syndromes. Pituitary adenomas (PA) are usually sporadic, but can be observed in MEN type 1 and in other genetic syndromes such as Carney’s complex syndrome, MEN type 4 (MEN4), and some hereditary pheochromocytomas/paragangliomas syndromes (HPPS).

Our aim was to analyze PA’s frequency and characteristics in aforementioned syndromes observed in our department.

Material and Methods: The retrospective study analyzed frequency and characteristics of PA associated to typical and atypical MENs. We excluded incomplete files and MEN2, as research for PA was not systematic, and we looked for MEN2 in PA too. The patients did not undergo genetic testing.

Results: Among ten hereditary syndromes (six MEN1 and four atypical MENs: either NEM4 or HPPS), we observed eight PA =80%. Four PA were associated with MEN1 and four with non-classical MENs. PA revealed MENs in four cases. PA median height was 19.88mm (7-50). Six/eight were macro tumors (≥1cm in height), with cavernous sinus invasion in two. PA types were prolactinomas (two), somatotroph adenomas (two), gonadotroph adenoma (one) and non-secreting tumors (three). For the follow up, only two out of six treated patients achieved remission.

Conclusion: In this series, PA were frequent (80%) in MEN1 and non-classical MENs. Macro adenomas and secreting tumors were prevailing with a variable aggressiveness. These syndromes genetically determined should have systematic genetic screening to specify MENs types and for rapid diagnosis and early management of affected patients and their families.

Keywords: Pituitary adenoma, MEN1, MEN4, Carney complex, Pheochromocytoma/paraganglioma.
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