Safety of long-term treatment with Pegvisomant: analysis of Spanish patients included in global ACROSTUDY.

PURPOSE: To evaluate the long-term safety of Pegvisomant (PEG) in the Spanish cohort of ACROSTUDY. METHODS: As of July 2013, 199 Spanish patients were included in ACROSTUDY, a global non interventional safety PEG surveillance study. Patients were observed for safety, biochemical outcome and magnetic resonance imaging evaluations. RESULTS: PEG was administered during an average period of 6.7 ± 2.1 years and a mean daily dose of 15.5 ± 7.5 mg. 48.2% of patients received PEG monotherapy. 90.9% of patients had received other medical treatment before PEG start. 195 adverse events (AEs) were reported in 88 patients (44.2%), and serious AEs were described in 31 patients (15.6%). There were no cases of liver tests >10 ULN, or permanent liver damage. Tumor size changes were locally reported in 61 cases (33.5%), with increases observed in 11 patients (6%). In acromegalic patients with diabetes mellitus a decrease in fasting serum glucose value was reported, reaching statistical significance after 1 and 4 years of treatment (-24.6 and -25.9 mg/dl, p = 0.04). After 60 months, normal or lower limit of normal (LLN) IGF-I levels were found in 67.9% of patients. 85.5% of patients showed an IGF-I normal or

Familial isolated pituitary adenoma caused by a Aip gene mutation not described before in a family context.

The cause of familial isolated pituitary adenomas (FIPA) remains unknown in a high percentage of cases, but the AIP gene plays an important role in the etiology. The aim of the study is to describe a family with FIPA syndrome and the results of genomic studies. A 16-year-old man had a giant prolactinoma resistant tomedical treatment with delayed growth and pubertal development. His mother had been previously diagnosed with a nonfunctioning pituitary macroadenoma. Transsphenoidal endoscopic resection was performed and a genetic study revealed a heterozygous mutation in exon 6: 974G>A (p.Arg325Gln). Because the AIP gene is a tumor suppressor gene, we searched for loss of heterozygosity within the AIP gene by amplifying exon 6 from tumor tissue of the patient. In the electropherogram, only the A allele was amplified (hemizygous state), indicating loss of the normal allele. We report a Spanish family with FIPA in whom a mutation in the AIP gene previously unreported in a familiar context was identified.

[Computerized tomography of the pituitary gland in the diagnosis of prolactinoma: a concordance study]. / La tomografía computadorizada de hipófisis en el diagnóstico de los prolactinomas: estudio de concordancia.

BACKGROUND: The diagnostic capacity of computed tomography (CT) of the pituitary gland in the diagnosis of prolactinomas is difficult to define in terms of specificity and sensitivity since, up to the present, there is no definite diagnostic test for prolactin producing tumors. The aim of this study is to establish the consistency of CT of the hypophysis in the diagnosis of the prolactinomas based on a concordance design. METHODS: In the follow-up study of 48 patients diagnosed as affected of prolactinoma a concordance study was carried out on the blind lecture of 35 pituitary gland CT by two radiologists. RESULTS: The degree of concordance for all the diagnosis (kappa = 0.58) was greater than what might be expected by chance. The degree of concordance was also different for the different diagnosis given by the two radiologists: empty sella turcica, kappa = 0.84 (p less than 0.01); macroprolactinoma, kappa = 0.68 (p less than 0.01); microprolactinoma, kappa = 0.45 (p less than 0.01) and normal sella turcica, kappa = 0.28 (NS). CONCLUSIONS: CT of the hypophysis must be less sensitive (more false negatives) in cases of high prolactin due to microprolactinomas and less specific (more false positives) in cases of high prolactin due to a cause other than pituitary tumor, thereby making it a complementary diagnostic test to clinical evaluation and to prolactin determinations.