The Nelsons syndrome (NS) is characterized by hyperpigmentation, sellar growing mass, and increased plasma ACTH concentrations The management of NS remains difficult: neurosurgery represent the first choice therapy. Many drugs have been used with variable results: cyproeptadine, GABA-agonists, somatostatin analogues, chemiotherapy, PPAR-γ-agonists, and dopamin-agonists.
Aim of this study was to evaluate the efficacy of cabergoline administration to reduce ACTH concentrations, hyperpigmentation and tumors size in NS.
Four women (2859 years, mean age 47±17 years) with NS, without aggressive or invasive course, were enrolled between May 2007 and July 2008. All patients were on replacement therapy with cortone acetate at the daily dose of 37.5 mg. All patients before the enrolment showed intrasellar persistent residual tissue (main diameter <1 cm) at MRI; two of them (patient no. 1, patient no. 2) underwent to radiosterotactic surgery 5 and 7 years before the enrollment respectively. In all patients the starting dose was 1 mg/week in two administrations and the dosage was increased every 3 months. All patients underwent every 3 months to clinical and hormonal assessment. Every 6 months pituitary MRI was performed. To exclude valvular abnormalities all patients were submitted to cardiac ecocolordoppler basally and every 12 months. Preliminary data showed a decrease of ACTH levels before and 2 h after corticosteroid therapy in two patients (patient no. 1 and 2; percentage of reduction was 68.6 and 81.4%, respectively), while in the other two patients (patient no. 3 and 4) we observed an increase (percentage of increase was 41 and 35.7%, respectively). We observed an improvement of hyperpigmentation in 2/4 patients (patient no. 1 and 2). During follow-up MRI didnt show any change with respect to time of enrollment in all patients.
These data suggest that cabergolin therapy could be useful to reduce ACTH concentrations and to prevent regrowth or increase of ACTH secreting adenomas in patients with NS previously treated with radiosterotactic surgery.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology