Recent changes in clinical presentation and therapeutic approach in MEN1-related pituitary adenomas
Valeria Ramundo, Rosa Severino, Francesco Milone, Gaetano Lombardi, Annamaria Colao & Antongiulio Faggiano
Background and objective: Pituitary adenomas (PAs) affect about half of the patients with multiple endocrine neoplasia type 1 (MEN1); in the most of cases PA is a PRL-oma and generally more aggressive than the sporadic form. The aim of this study is to evaluate the recent changes in clinical presentation and therapeutic approach of MEN1-related PAs.
Patients and methods: The study population included 17 patients with MEN1-related PA followed-up between 1990 and 2010. The whole study population included 8 PRL-omas, 2 GH-omas (associated with PRL secretion in one), 7 non-functioning adenomas. Patients were divided in two groups: group A (PA diagnosed before 2008, 9 patients) and group B (PA diagnosed in 20082010, 8 patients).
Results: The prevalence of macroadenoma was 67% in group A and and 37% in group B. PA-related symptoms were present in 67% of patients of group A and 50% of patients of group B. PA was treated in 78% of patients group A (2 transsphenoidal surgery, 5 medical therapy) and 50% of patients group B (4 medical therapy). Medical therapy included cabergoline for PRL-omas, somatostatin analogues for GH-oma and both for GH-PRL adenoma. Both group A patients treated with transsphenoidal surgery had postoperative tumor persistence. A significant hormone response was observed in all cases of medically-treated patients of group A and in 75% of medically-treated patients of group B. At the pituitary MRI, tumor shrinkage was observed in 33% of cases in group A and 50% in group B.
Conclusions: Clinical presentation of MEN1-related PA is changed in the last years, resulting in early diagnosed and less aggressive tumors. For these reasons and for the availability of effective medical compounds, transsphenoidal surgery is now less necessary and medical therapy with cabergoline and/or somatostatin analogues is the main therapeutic approach.