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Objectives: To analyse the prognostic value of telomerase expression in patients with pituitary adenomas (PAs) followed-up for at least 8 years., Patients and Methods: A retrospective study was conducted of samples from 51 PAs (40 typical and 11 atypical) from patients who underwent transsphenoidal surgery between 2006 and 2008 and from 10 normal pituitary glands obtained by autopsy. Telomerase expression was assessed by immunohistochemistry, correlating the expression with that of Ki-67 and p53., Results: We observed telomerase expression in 43 PAs (84.3%, 32 of the 40 typical PAs and in the 11 atypical PAs), which was higher in the clinically nonfunctioning cases (P=.0034) and very rare in the patients with acromegaly (P=.0001). There was a significant association between the percentage of tumour cells (>10%) and the recurrence of the adenoma (P=.039). There was no correlation with the expression of Ki-67 and p53 (P=.4986), and there were no differences according to age, sex, tumour size and invasiveness., Conclusions: A telomerase expression rate greater than 10% in the pituitary tumour tissue was associated with recurrence or progression of the PA, especially in the nonfunctioning cases., (Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.)

The sellar and parasellar region is a complex anatomical area in which several diseases may develop. The pituitary gland may be affected by a wide range of conditions having similar clinical characteristics. Diagnosis of these lesions requires a multidisciplinary approach including, in addition to clinical, laboratory, imaging, and surgical findings, histological diagnosis of pituitary adenomas to guide therapeutic management. As the result of development in recent years of new immunohistochemical techniques, histopathological classification has become more complex and wide, and not only continues to be the gold standard in diagnosis, but also has prognostic implications. The aim of this review is to provide a clear and simple update of the main concepts of histological diagnosis of the most common pituitary conditions, especially for professionals in direct contact with such diseases., (Copyright © 2016 SEEN. Publicado por Elsevier España, S.L.U. All rights reserved.)

Pituitary adenomas are uncommon, difficult to diagnose tumors whose heterogeneity and low incidence complicate large-scale studies. The Molecular Registry of Pituitary Adenomas (REMAH) was promoted by the Andalusian Society of Endocrinology and Nutrition (SAEN) in 2008 as a cooperative clinical-basic multicenter strategy aimed at improving diagnosis and treatment of pituitary adenomas by combining clinical, pathological, and molecular information. In 2010, the Spanish Society of Endocrinology and Nutrition (SEEN) extended this project to national level and established 6 nodes with common protocols and methods for sample and clinical data collection, molecular analysis, and data recording in a common registry (www.remahnacional.com). The registry combines clinical data with molecular phenotyping of the resected pituitary adenoma using quantitative real-time PCR of expression of 26 genes: Pituitary hormones (GH-PRL-LH-FSH-PRL-ACTH-CGA), receptors (somatostatin, dopamine, GHRH, GnRH, CRH, arginine-vasopressin, ghrelin), other markers (Ki67, PTTG1), and control genes. Until 2015, molecular information has been collected from 704 adenomas, out of 1179 patients registered. This strategy allows for comparative and relational analysis between the molecular profile of the different types of adenoma and the clinical phenotype of patients, which may provide a better understanding of the condition and potentially help in treatment selection. The REMAH is therefore a unique multicenter, interdisciplinary network founded on a shared database that provides a far-reaching translational approach for management of pituitary adenomas, and paves the way for the conduct of combined clinical-basic innovative studies on large patient samples., (Copyright © 2016 SEEN. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: Primary pituitary tumours are classified by the World Health Organization as typical adenoma, atypical adenoma, or carcinoma. Information on the incidence and prevalence of these pituitary tumours is limited, and these data in Portugal are scarce, obsolete, or non-existent. Our study evaluates pituitary adenomas (PA) in the population of Lisbon, and it aims to describe the prevalence of all subgroups in order to revise the incidence of the 'atypical' histological type and its correlation to tumour subtype, invasion, and recurrence., Patients and Methods: A retrospective, descriptive analysis of patients with PA diagnosed between 2004 and 2013 was performed at Santa Maria University Hospital, a national reference centre., Results: Of the 220 PA cases diagnosed, 28 (12.7%) fulfilled criteria for atypical lesions, and within that group, 23 were macroadenomas (82.1%) and 13 showed radiological evidence of invasion (46.4%). Ages ranged from 29 and 81 years (mean, 53.4 years). Eleven patients (39.3%) had functional tumours. Sixteen of the 28 patients (57.1%) experienced tumour recurrences; in the 100 adenomas monitored for more than 5 years, the recurrence rate in atypical PA was 7 times higher than in typical PA. Immunohistochemically, 28.6% of the tumours stained positively for ACTH, 25% for gonadotrophins, and 17.9% for prolactin. The proliferation index (Ki67) ranged from 3% and 25% (mean, 6.4%)., Conclusions: Atypical PAs make up 12.7% of all surgically treated PA cases, and they tend to be invasive and recurrent macroadenomas. We found no differences in metastatic potential between typical and atypical PA., (Copyright © 2015 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Cushing syndrome (CS), due to an ACTH-secreting pituitary adenoma, adrenal tumors, or ectopic ACTH secretion, causes hypercortisolism. CS is associated with major morbidity, especially metabolic and cardiovascular complications, osteoporosis, psychiatric changes, and cognitive impairment. Despite biochemical "cure" of hypercortisolism and clinical improvement after effective treatment, these complications are only partially reversible. Exacerbation of prior autoimmune diseases is also seen. All of these lead to quality of life impairment and increased mortality. This review addresses the main comorbidities and long-term consequences of CS despite clinical and biochemical "cure"., (Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.)

Cushing's syndrome is due to excess cortisol secretion and is associated to increased mortality and severe morbidity that are not fully reversible despite biochemical control. The syndrome consists of a set of systemic manifestations similar to those found in aging. Chronic stress, which also causes hyperstimulation of the hypothalamic-pituitary-adrenal axis, has been related to accelerated telomere shortening, oxidative damage, and cell aging. Although premature aging in patients with Cushing's syndrome could be related to environmental factors, the possibility that chronic exposure to hypercortisolism causes telomere shortening, and thus premature aging, cannot be ruled out. This review discusses the available evidence supporting a link between Cushing's syndrome and cell aging., (Copyright © 2012 SEEN. Published by Elsevier Espana. All rights reserved.)

Introduction: A multidisciplinary team is essential to develop and expand the indications in endonasal endoscopic skull base surgery. The aim of this study was to present our experience in a group of patients with skull base lesions treated using endonasal endoscopic approach., Methods: From January 2008 to January 2012, 72 patients with skull base involvement were diagnosed and treated in our centre., Results: The mean patient age was 53 years. The different pathologies included 36 pituitary adenomas, 10 cerebrospinal fluid leaks and 5 inverted papillomas as the most frequent pathologies. We performed a transsphenoidal transellar approach in 45 cases, a transmaxillary transpterygoid approach in 4 cases and a transnasal expanded approach in 6 cases. We performed an ethmoidal/sphenoidal approach in 12 patients and a Draf IIb/III procedure in four cases. Total resection was achieved in 61% of patients with pituitary adenomas, subtotal in 22% and partial in 17%. Successful repair was achieved in 86% of CSF leaks. No recurrences were observed in patients with inverted papilloma. Complications were observed in 21 patients (29%), 6 being major complications., Conclusions: Our centre stresses the importance of multidisciplinary collaboration in endoscopic endonasal skull base surgery., (Copyright © 2012 Elsevier España, S.L. All rights reserved.)

Adrenal insufficiency (AI) is a rare endocrine disease, associated to increased mortality if left untreated. It can be due to a primary failure of the adrenal glands (primary AI) or malfunctioning of the hypothalamic-pituitary-adrenal axis (HPA) (secondary AI). The lack of data on incidence/prevalence of adrenal insufficiency in Spain complicates any evaluation of the magnitude of the problem in our country. Initial symptoms are non-specific, so often there is a delay in diagnosis. Current therapy with available glucocorticoids is associated with decreased quality of life in patients with treated AI, as well as with increased mortality and morbidity, probably related to both over-treatment and lack of hydrocortisone, associated with non-physiological peaks and troughs of the drug over the 24 hours. The availability of a new drug with a modified dual release (immediate and retarded), that requires one only daily dose, improves and simplifies the treatment, increases compliance as well as quality of life, morbidity and possibly mortality. This revision deals with the knowledge on the situation both globally and in Spain, prior to the availability of this new drug., (Copyright © 2012 SEEN. Published by Elsevier Espana. All rights reserved.)

The incorporation of subjective elements provided by the patient to medical practice constitutes a new bridge between two historical movements of human culture: naturalism and rationalism. In recent years, medical sciences have identified these movements as patient-centered medicine and evidence-based medicine. By revisiting the paradigms of medicine, theories of causality, doctor-patient relationship models, bioethical principles and examples of clinical studies, their role and meaning has been revised. The applicability for neuroendocrinology has been demonstrated by validating therapeutic results with measurement of health-related quality of life. The need to incorporate the patient's subjective perception poses a challenge that, when accepted, can advance the understanding and care of human beings--ultimately rational, thinking subjects, and therefore subjective--as our species has been defined.

Inadequate secretion of TSH (IST) is a disorder which is diagnosed more frequently and earlier after the introduction of new immunoassay techniques which can distinguish between normal and suppressed TSH levels. For diagnosis high or unsuppressed TSH in required in the presence of elevated levels of the thyroid hormones. Its etiology may be tumor (TSH secreting pituitary adenoma) or non tumoral due to pituitary or generalized resistance to the thyroid hormones. Differential diagnosis between both etiologies is not easy, and several tests have been proposed but are not always discriminatory. Five cases of IST are presented in whom the diagnostic, clinical and therapeutic criteria have been analyzed. The cases of neoplastic IST (patients n.o 3, 4, and 5) showed a loss in circadian rhythm of TSH and absence of suppression with triiodothyronine (T3), 3,5-diiodo 4-(3'-iodine 4'-hydroxyphenoxi) phenylacetic acid (TRIAC) and with bromocriptine. The circadian rhythm of TSH was maintained in the non neoplastic IST (patients n.o 1 and 2) as was suppressed with T3, TRIAC and bromocriptine. The subunit-alpha/TSH quotient and TSH response to TRH were variable with no stimulation being observed with metoclopramide in any case. Upon the demonstration of unsuppressed circulating TSH in the presence of biochemical hyperthyroidism, IST should be suspected to avoid erroneous diagnosis and treatments. Differentiation between neoplastic and non neoplastic origin may be difficult since the biochemical and neuropharmacologic parameters are not always discriminatory.

The clinical, hydroelectrolytical and hormonal features, as well as the treatment of 3 patients with Bartter's syndrome are analyzed. The patients consulted due to severe hypokalaemia with little clinical expression (paresthesiae, carpopedal spasms, polyuria, polydipsia and/or weakness). All had normal blood pressure, hypokalemia, hyperreninaemic hyperaldosteronism and a decrease in the fractional distal chloride reabsorption; hyperuricaemia was observed in two cases and hypomagnesemia in one. Treatment with oral potassium supplements and indomethacin managed to raise serum K to around the lower limit of the normal range. However, the tubular defect and hyperreninaemic hyperaldosteronism persisted. The urinary excretion of prostaglandin E2 was normal in both the patients in whom it was measured. Given the minimal clinical expression of hypokalemia despite the difficulty in correcting it, it is probable that this syndrome often remains unrecognized.

The results of the treatment of acromegaly in 56 patients have been reviewed. Transsphenoidal pituitary adenomectomy (TPA) represented the most effective therapy, as it resulted in a cure rate of 64.7%, with 8.8% of complications and 14.7% of endocrine insufficiencies secondary to surgery. When radiotherapy was administered after surgery (when the latter had not been curative), the overall rate of cure increased to 73.5%. By contrast, the patients treated only with radiotherapy had a markedly lower cure rate (44.4%), with a higher rate of endocrine insufficiency (55%). The occurrence of extrasellar extension shown in preoperative pituitary computed tomography did not correlate with a worse postoperative cure rate; by contrast, the invasive character of the adenoma represented a reduction in the postoperative cure rate. It was concluded that TPA is the treatment of choice in acromegaly, independently of the tumor size.

A female is reported with a pituitary tumor secreting growth hormone which remained active and induced invalidating headache in spite of previous treatment with surgery and radiotherapy. Treatment with the sustained action somatostatin analogue, SMS 201-995, was started and headache was improved in a matter of minutes, even if normalization of hormone hypersecretion was not demonstrated. The pathophysiological mechanisms possibly implicated in the improvement are discussed, and this therapeutic option in patients with headache unresponsive to common analgesics is emphasized.