The Mexican Acromegaly Registry: Clinical and Biochemical Characteristics at Diagnosis and Therapeutic Outcomes.

CONTEXT: Acromegaly is a systemic disorder caused by a GH-secreting pituitary adenoma. As with other rare diseases, acromegaly registries developed in various European countries have provided us with important information. OBJECTIVE: The objective of the study was to analyze the epidemiological, clinical, biochemical, and therapeutic data from the Mexican Acromegaly Registry (MAR). SETTING: The setting of the study was a nationwide patient registry. DESIGN AND METHODOLOGY: The MAR was created in 2009. It gathers data from 24 participating centers belonging to three different institutions using a specifically designed on-line platform. Only patients diagnosed after 1990 were included in the program. RESULTS: A total of 2057 patients (51% female, mean age at diagnosis 41.1 ± 24.5 y) have been registered for an estimated prevalence of 18 cases per 1 million inhabitants. Hypertension, glucose intolerance, diabetes, and dyslipidemia were present in 27%, 18.4%, 30%, and 24% of the patients, respectively. The IGF-1 level at diagnosis and the concomitant presence of hypertension were significantly associated with the development of diabetes. Transsphenoidal surgery was the primary treatment in 72% of the patients. Pharmacological treatment, mostly with somatostatin analogs, was administered primarily and adjunctively in 26% and 54% of the patients, respectively. Treatment choice varied among the three participating institutions, with the predominance of pharmacological therapy in two of them and of radiation therapy in the third. Therapeutic outcomes were similar to those reported in the European registries. CONCLUSIONS: The MAR is the largest and first non-European registry of the disease. Our findings highlight important within-country differences in treatment choice due to variations in the availability of resources.

Novel pathway for somatostatin analogs in patients with acromegaly.

Acromegaly is a chronic disease with increased morbidity and mortality, where usually multiple treatment modalities are used. The somatostatin analogs (SSAs) are the mainstay of medical therapy but, in many patients, including those with a germline mutation in the aryl hydrocarbon receptor-interacting protein (AIP) gene, disease activity cannot be controlled with these drugs. Previous data have suggested the involvement of the tumor-suppressor gene ZAC1 in the mechanism of action of SSAs, and more recent findings suggested that SSAs could regulate AIP, which in turn can stimulate ZAC1, therefore suggesting the existence of a SSA-AIP-ZAC1-somatostatin effect pathway. The current review discusses these novel observations, highlighting their significance in the treatment of sporadic and familial somatotroph adenomas.

Outcome of surgical intrasellar growth hormone tumor performed by a pituitary specialist surgeon in a developing country.

BACKGROUND: Acromegaly is an excessive GH secretion, which in most cases, is caused by a pituitary GH-secreting adenoma. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The aim of this retrospective study is to evaluate the results of transsphenoidal endoscopic surgery in a group of patients with intrasellar GH adenoma who were operated by a pituitary specialist surgeon. We shall then argue about the economical advantages, for the NHS of a developing country, between surgical and medical treatment. METHODS: We have analyzed data from 33 patients with intrasellar GH tumor who had been referred to the neuroendocrine department of the HGF, Brazil. The patients underwent a transsphenoidal endoscopic adenomectomy for acromegaly between 2000 and 2005. Their ages were between 20 and 67 years (mean, 44 years) at the moment of surgery. No cavernous sinus invasion was present. Follow-up was a median of 2 years (range, 12 months-6 years). RESULTS: All 33 patients had intrasellar adenoma, 84.84% of patients achieved remission by surgery. One patient was operated twice and reached hormonal normalization. Five patients still had the disease and refused a second surgery. A treatment with octreotide was started for these 5 patients and resulted in an adequate control of GH and IGF-1 levels. No patients had radiotherapy. CONCLUSION: Our patients, with intrasellar GH tumor, operated by a pituitary specialist neurosurgeon had remission rates approaching those obtained by most specialized neurosurgical centers worldwide. For equal results, our study shows that the surgical treatment is the best issue for the patient and for the NHS.