Gigantism: microsurgical treatment by transsphenoidal approach and prognostic factors.

PURPOSE: We present the results of transsphenoidal microsurgical treatment in 14 patients with gigantism. The influence on the prognosis of factors such as the tumor size and preoperative levels of GH and IGF-1 is also quantified. MATERIALS AND METHODS: The patients, operated between 1982 and 2004, were reviewed retrospectively in June 2022. All patients had complete endocrinological studies in the preoperative period and a postoperative control between 6 days and 3 weeks. Follow-up has been supported with annual check-ups between 3 and 31 years. We have compared the preoperative levels of GH and IGF-1 of these patients with the levels of a series of acromegalic patients operated on in the same Center. RESULTS: In this series there were 4 women and 10 men. The age ranged between 14 and 21 years. In 6 patients, postoperative hormone levels achieved the disease control criteria (42.8%). The CT/MRI studies revealed the existence of invasive tumors in 10 of the patients (71.4%). Postoperative CT/MRI showed no tumor tissue in 3 patients but in 7 patients there were tumor remains. The remaining 4 patients had abnormal images although not considered as tumor. A statistical comparison of preoperative serum GH and IGF-1 levels in patients with gigantism and patients with acromegaly showed a significant elevation in the former. CONCLUSION: Pituitary adenomas that cause gigantism are generally large and invasive, which makes them difficult to cure. High preoperative levels of GH and IGF-1 are also factors that decrease remission.

El Registro Molecular de Adenomas Hipofisarios (REMAH): una apuesta de futuro de la Endocrinología española por la medicina individualizada y la investigación traslacional / The Molecular Registry of Pituitary Adenomas (REMAH): A bet of Spanish Endocrinology for the future of individualized medicine and translational research

Los adenomas hipofisarios son tumores infrecuentes de diagnóstico complejo, cuya heterogeneidad y baja incidencia dificultan estudios a gran escala. El Registro Molecular de Adenomas Hipofisarios (REMAH) nació en 2008 en el seno de la Sociedad Andaluza de Endocrinología y Nutrición (SAEN), como estrategia de cooperación clínico-básica y multicéntrica, para mejorar el diagnóstico y tratamiento de tumores hipofisarios mediante la combinación de información clínica, anatomopatológica y molecular. En 2010, la Sociedad Española de Endocrinología y Nutrición (SEEN) lo extendió a nivel nacional, estableciendo 6 nodos con protocolos y métodos comunes de recogida de muestras y datos clínicos, análisis molecular y anotación en un mismo registro (www.remahnacional.com). El registro combina datos clínicos con el fenotipado molecular del adenoma intervenido, mediante PCR cuantitativa en tiempo real de la expresión de 26 genes: hormonas hipofisarias (GH-PRL-LH-FSH-PRL-ACTH-CGA), receptores (somatostatina, dopamina, GHRH, GnRH, CRH, arginina-vasopresina, ghrelina), otros marcadores (Ki67, PTTG1) y genes de control. Hasta 2015 se ha obtenido información molecular de 704 adenomas, de los 1.179 pacientes registrados. Esta estrategia permite abordar análisis comparativos y relacionales entre el perfil molecular de los distintos tipos de adenomas y el fenotipo clínico del paciente, lo que puede ofrecer un mejor conocimiento de la enfermedad y, potencialmente, ayudar en la selección del tratamiento. El REMAH constituye una red única, multicéntrica e interdisciplinar, cimentada en una base de datos compartida, que aporta un enfoque traslacional de gran proyección potencial para el manejo de los adenomas hipofisarios y abre el camino para estudios conjuntos clínico-básicos innovadores con un elevado número de pacientes (AU)

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 (AU)

The Molecular Registry of Pituitary Adenomas (REMAH): A bet of Spanish Endocrinology for the future of individualized medicine and translational research. / El Registro Molecular de Adenomas Hipofisarios (REMAH): una apuesta de futuro de la Endocrinología española por la medicina individualizada y la investigación traslacional.

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.

[Acromegalic myocardiopathy]. / Miocardiopatía acromegálica.

Acromegaly is characterized by chronic growth hormone hypersecretion. Cardiovascular alterations such as hypertension, left ventricular hypertrophy, cardiac rhythm disturbances and valvular disease are common in this disease and are the main cause of death. Control of acromegaly by surgery or pharmacotherapy has been shown to improve cardiovascular morbidity. We report a case of acromegalic myocardiopathy in a 59-year-old woman with dilated myocardiopathy who presented ventricular diameter and contractility normalization following medical treatment.

Miocardiopatía acromegálica / Acromegalic myocardiopathy

La acromegalia es una enfermedad caracterizada por la hipersecreción crónica de hormona de crecimiento. Alteraciones cardiovasculares, como hipertensión, hipertrofia ventricular izquierda, alteraciones del ritmo cardíaco y valvulopatías son frecuentes en esta enfermedad y representan la principal causa de mortalidad. El control de la acromegalia mediante cirugía o tratamiento médico ha demostrado mejorar la morbilidad cardiovascular. Presentamos el caso de una miocardiopatía acromegálica en una mujer de 59 años con una miocardiopatía dilatada que presentó normalización del diámetro y la contractilidad ventricular tras tratamiento médico (AU)

Acromegaly is characterized by chronic growth hormone hypersecretion. Cardiovascular alterations such as hypertension, left ventricular hypertrophy, cardiac rhythm disturbances and valvular disease are common in this disease and are the main cause of death. Control of acromegaly by surgery or pharmacotherapy has been shown to improve cardiovascular morbidity. We report a case of acromegalic myocardiopathy in a 59-year-old woman with dilated myocardiopathy who presented ventricular diameter and contractility normalization following medical treatment (AU)

Clinical practice guideline for hypotalamic-pituitary disturbances in pregnancy and the postpartum period.

During pregnancy, the body undergoes a major adaptation process as a result of the interaction between mother, placenta and fetus. Major anatomical and histological changes are produced in the pituitary, with an increase of up to 40% in the size of the gland. There are wide variations in the function of the hypothalamus-pituitary-thyroid axis that effect iodine balance, the overall activity of the gland, as well as transport of thyroid hormones in plasma and peripheral metabolism of thyroid hormones. The incidence of goiter and thyroid nodules increases throughout pregnancy. The management of differentiated thyroid carcinoma should be individually tailored according to tumoral type and pregnancy stage. Given the effects of hypothyroidism on fetal development, both the diagnosis and appropriate therapeutic management of thyroid hypofunction are essential. The most important modification to the hypothalamus-pituitary-adrenal axis during pregnancy is the rise in serum cortisol levels due to an increase in cortisol-binding proteins. Although Cushing's syndrome during pregnancy is infrequent, both diagnosis and treatment of this disorder are especially difficult. Adrenal insufficiency during pregnancy does not substantially differ from that occurring outside pregnancy. However, postpartum pituitary necrosis (Sheehan's syndrome) is a well-known complication that occurs after delivery and, together with lymphocytic hypophysitis, constitutes the most frequent cause of adrenal insufficiency. The management of prolactinoma during pregnancy requires suppression of dopaminergic agonists and their reintroduction if there is tumoral growth. Notable among the neuropituitary disorders that can occur throughout pregnancy is diabetes insipidus, which occurs as a consequence of increased vasopressinase activity.

Clinical practice guideline for the diagnosis and treatment of hypophysitis.

Hypophysitis are a group of inflammatory lesions affecting the pituitary gland and pituitary stalk. These lesions should be included in the differential diagnosis of sellar masses. There are three types of primary hypophysitis: lymphocytic, granulomatous and xanthomatous. Lymphocytic hypophysitis is the most frequent form of chronic pituitary inflammation and is believed to have an autoimmune origin. This form characteristically affects women during the peripartum, with diverse types of pituitary deficiency, especially ACTH deficiency, and frequently there are other associated autoimmune processes. Lymphocytic hypophysitis can affect the anterior pituitary only, the infundibular stalk and posterior lobe of the pituitary (infundibuloneurohypophysitis), or the entire pituitary (panhypophysitis). Clinically, lymphocytic hypophysitis can manifest with compression symptoms, hypopituitarism, diabetes insipidus or hyperprolactinemia. The imaging technique of choice is magnetic resonance imaging, which helps to characterize the sellar lesion. Treatment includes replacement of the functional pituitary deficiency and the use of corticosteroids, generally at high doses. Surgical treatment is reserved for patients unresponsive to conservative therapy. Granulomatous hypophysitis can be of known etiology, whether infectious (currently highly infrequent) or non-infectious (ruptured Rathke's cyst, etc.). Granulomatous hypophysitis of unknown etiology is manifested by the presence of idiopathic granulomas. Xanthomatous hypophysitis is characterized by a histiocytic infiltrate with cystic characteristics on imaging. Secondary hypophysitis is due to pituitary inflammation caused by surrounding lesions or can form part of systemic diseases.

Guía clínica del diagnóstico y tratamiento del craneofaringioma y otras lesiones paraselares / Clinical practice guideline for the diagnosis and treatment of craniopharyngioma and other parasellar lesions

El craneofaringioma es un tumor, con frecuencia quístico, habitualmente supraselar, que deriva de restos de células embrionarias de la bolsa de Rathke. Si bien es un tumor benigno, tiene un comportamiento agresivo con frecuentes secuelas neurológicas y endocrinas. Presenta dos picos de aparición: en la edad infantil y en adultos añosos. La clínica depende de la localización, el tamaño, el potencial de crecimiento y la edad de presentación. Clínicamente suele aparecer como una combinación de signos y síntomas de hipertensión intracraneal, alteraciones visuales, deficiencias hormonales y disfunción hipotalámica. Si la lesión es intraselar la clínica puede remedar a la de un adenoma hipofisario. Las técnicas de neuroimagen, especialmente la resonancia magnética, permiten caracterizar la lesión. La apariencia varía dependiendo de la proporción del componente sólido y quístico, de las posibles calcificaciones y de la composición de un eventual quiste. Antes del abordaje terapéutico debe efectuarse una completa evaluación endocrinológica y oftalmológica. Las opciones terapéuticas incluyen cirugía, radioterapia y una combinación de ambas. La extensión óptima de la cirugía es motivo de controversia. Actualmente se prefiere una aproximación más conservadora que combina una cirugía menos agresiva con radioterapia. La radioterapia sin cirugía únicamente es aplicable a los pacientes con tumores muy pequeños. Otras aproximaciones incluyen: aspiración intermitente mediante punción esterotáxica, colocación de un reservorio, esclerosis de las paredes del quiste mediante fármacos, o irradiación interna con radioisótopos. Las lesiones paraselares son lesiones de muy baja prevalencia y pueden ser, entre otros tumores, aneurismas, quistes o granulomas. Las técnicas de neuroimagen, tanto la tomografía computarizada como la resonancia magnética, son útiles para precisar las características de la lesión (AU)

Craniopharyngiomas are often cystic tumors, usually suprasellar, resulting from embryonic cell remnants of Rathke’s pouch. Although benign, these tumors can be aggressive and frequently have neurological and endocrinological sequelae. Craniopharyngiomas usually develop in children or in the elderly. Symptoms depend on localization, size, potential for growth, and age of onset. Clinically, craniopharyngiomas usually manifest with a combination of symptoms and signs of intracranial hypertension, visual alterations, hormone deficiencies, and hypothalamic dysfunction. Intracellular lesions can mimic pituitary adenoma. Neuroimaging techniques, especially magnetic resonance imaging, allow these lesions to be characterized. Their appearance varies depending on the proportion of solid and cystic components, on the possible calcifications, and on the composition of an eventual cyst. Complete endocrinological and ophthalmological evaluation should be performed before establishing the therapeutic approach. The therapeutic options include surgery, radiotherapy, and a combination of both. The optimal extension of surgery is controversial. Currently, a conservative approach combining less aggressive surgery with radiotherapy is preferred. Radiotherapy without surgery is only applicable in patients with very small tumors. Other approaches include intermittent aspiration by stereotactic puncture, placement of a reservoir, cystic wall sclerosis through drugs, or internal radiation with radioisotopes. Parasellar lesions have a very low prevalence and can consist of cystic aneurysms or granulomas, among other tumors. Neuroimaging techniques, both computed tomography and MRI, are useful for characterizing the lesion (AU)

Guía clínica del diagnóstico y tratamiento de los trastornos de la neurohipófisis / Clinical practice guideline for the diagnosis and treatment of neuropituitary disorders

La homeostasis del agua corporal se halla regulada por la ingesta de agua que depende principalmente de la sed y de la excreción urinaria modulada, fundamentalmente, por la vasopresina (AVP). En ausencia de AVP el túbulo colector es impermeable a la difusión de agua, dando lugar a una diuresis acuosa o diluida con osmolalidades urinarias menores a 100 mOsm/kg. Por el contrario, en presencia de AVP, la permeabilidad se incrementa considerablemente, el agua es reabsorbida libre de solutos y la orina alcanza osmolalidades superiores a 1.000 mOsm/kg. La diabetes insípida es el síndrome resultante de la alteración corporal del agua debido a una deficiencia en la secreción de AVP (diabetes insípida central o neurogénica) o por falta de acción de la AVP en el túbulo colector del riñón (diabetes insípida nefrogénica). El síndrome se caracteriza por poliuria con eliminación de grandes volúmenes de orina (> 3,5 l/día), polidipsia y síntomas de tipo general. Respecto a la etiología, en la diabetes insípida central existen formas familiares, y con mucha mayor frecuencia formas adquiridas: tras cirugía hipotálamo-hipofisaria o traumatismos craneoencefálicos, tumores, granulomas, idiopáticas y otras. La diabetes insípida nefrogénica puede estar producida, a su vez, por causa genéticas o familiares o adquiridas secundarias a fármacos, alteraciones metabólicas y otras. Las pruebas diagnósticas en los estados poliúricos incluyen un estudio basal con determinaciones simultáneas de la osmolalidad plasmática y urinaria, y si éstas no son concluyentes, la prueba de deprivación del agua o prueba de la sed, que permite discernir entre el cuadro de potomanía, y la diabetes insípida, bien central o bien nefrogénica. El diagnóstico de una diabetes insípida neurogénica obliga a la realización de una resonancia magnética hipotálamo-hipofisaria y al estudio hormonal de la hipófisis anterior. El análogo de la vasopresina, la desmopresina, es el tratamiento de elección de la diabetes insípida central. El síndrome de secreción inadecuada de hormona antidiurética (SIADH) es el cuadro derivado de la secreción no fisiológica de AVP y se caracteriza por la presencia de hiponatremia debida a la disminución en la excreción de agua libre. El síndrome puede estar producido por tumores, procesos neurológicos, pulmonares y fármacos. El diagnóstico se realiza con la demostración de hiponatremia, con hipoosmolalidad plasmática, osmolalidad urinaria elevada y ausencia de estados de depleción de volumen, hipervolémicos o insuficiencia renal, así como función tiroidea y adrenal normal. El tratamiento se basa en la restricción hídrica en casos de hiponatremia leve y moderada, precisando aporte de suero salino hipertónico en caso de hiponatremia grave (AU)

Body fluid homeostasis is regulated by water intake, which depends mainly on thirst and urine excretion mainly modulated by arginine vasopressin (AVP). In the absence of AVP, the collecting tubule is impermeable to water diffusion, giving rise to water diuresis with a urinary osmolality of less than 100 mOsm/Kg. In contrast, in the presence of AVP, permeability is considerably increased, water is reabsorbed free of solutes, and urinary osmolality is above 1000 mOsm/Kg. Diabetes insipidus results from an alteration in body water due to inadequate AVP release (central or neurogenic diabetes insipidus) or to a lack of AVP activity in the renal collecting tubule (nephrogenic diabetes insipidus). The syndrome is characterized by polyuria with excretion of large volumes of urine (>3.5 L/day), polydipsia, and general symptoms. The etiology of central diabetes insipidus can be familial but this disease is more frequently caused by acquired forms after hypothalamic-pituitary surgery, head injuries, tumors, granulomas, and idiopathic and other forms. Nephrogenic diabetes insipidus can be produced by genetic, familial, or acquired forms secondary to drugs, metabolic alterations, and other factors. Diagnostic tests in polyuric states include baseline evaluation with simultaneous determination of plasma and urinary osmolality and, if these tests are inconclusive, the water deprivation test allows symptoms of potomania and diabetes insipidus, whether central of nephrogenic, to be distinguished. Diagnosis of neurogenic diabetes insipidus requires hypothalamic-pituitary magnetic resonance imaging and hormonal study of the anterior pituitary gland. The treatment of choice for central diabetes insipidus is the vasopressin analog, desmopressin. The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) results from non-physiological AVP secretion and is characterized by the presence of hyponatremia due to impaired free water excretion. This syndrome can be caused by tumors, neurological processes, pulmonary disease, and drugs. Diagnosis is based on findings of hyponatremia with plasma hypoosmolality, elevated urine osmolality, absence of volume depletion states and hypervolemia, and normal renal, adrenal, and thyroid function. Treatment consists of water restriction in mild and moderate hyponatremia. Hypertonic saline is required in severe hyponatremia (AU)

Guía clínica del tratamiento hormonal sustitutivo de las deficiencias hormonales de la hipófisis anterior / Clinical practice guideline for hormone replacement therapy in anterior pituitary deficiencies

Una vez establecido el diagnóstico de deficiencia de corticotropina se lleva a cabo la sustitución de glucocorticoides, de preferencia con hidrocortisona. Tradicionalmente se emplean dosis de alrededor de 30 mg/día, repartidos en 2 dosis, pero los estudios de producción diaria y los valores plasmáticos de cortisol a lo largo del día indican mejores resultados con dosis más bajas y más fraccionadas. En la insuficiencia suprarrenal aguda se debe incrementar la dosis de hidrocortisona de forma proporcional a la situación de estrés hasta el equivalente a la secreción máxima de cortisol. La deficiencia de tirotropina (TSH) se debe suplir con L-tiroxina sódica aproximadamente 1,7 µg/kg/día. No debe iniciarse el tratamiento hasta haber evaluado la reserva adrenal. De igual forma que en el diagnóstico, la determinación de TSH no es útil para la monitorización del tratamiento. La dosis final de tiroxina dependerá de la normalización de la T4 libre. El tratamiento del hipogonadismo masculino tiene como objetivos restaurar la función sexual, mantener los caracteres sexuales secundarios y también la prevención de la osteoporosis. El tratamiento de elección del paciente con hipogonadismo hipogonadotropo es la testosterona. En la actualidad se dispone de parches transdérmicos o escrotales, geles transdérmicos y preparados intramusculares y de depósito. En aquellos casos en que se desee fertilidad debe iniciarse tratamiento con gonadotropinas; sólo si no hay respuesta se iniciará tratamiento con hormona liberadora de gonadotropina (GnRH). El tratamiento del hipogonadismo secundario en la mujer depende del objetivo que se persigue: tratamiento sustitutivo o inducción de la ovulación. En el primer caso se utilizarán preparados con estrógenos y progestágenos, bien por vía oral o a través de parches transdérmicos. Para la inducción de la ovulación deberán administrarse gonadotropinas varios meses o bien administración pulsátil GnRH si el déficit es hipotalámico (AU)

Once a diagnosis of adrenocorticotropic hormone (ACTH) deficiency has been made, glucocorticoid replacement therapy, preferably hydrocortisone, is indicated. Traditionally, a dosage of approximately 30 mg/day, divided into 2 doses, has been used. However, studies of plasma cortisol levels throughout the day indicate that better results are achieved with lower and more frequent doses. In adrenal crises, hydrocortisone dose should be increased in proportion to the stress until reaching the equivalent of maximal cortisol secretion. Thyrotropin deficiency should be treated with sodium L-thyroxine at a dosage of approximately 1.7 µg/Kg/day. Adrenal reserve should be evaluated before starting treatment. As in diagnosis, thyroid-stimulating hormone determination is not useful for treatment monitoring. The final thyroxin dosage will depend on normalization of free T4. The aim of treatment of male hypogonadism is to restore sexual function, maintain secondary sexual characteristics, and prevent osteoporosis. The treatment of choice in patients with hypogonadotropic hypogonadism is testosterone. Currently, transdermal or scrotal patches, transdermal gels, and intramuscular and depot injections are available. In patients wishing to preserve fertility, gonadotropin therapy should be started and, only if there is no response, should gonadotropin-releasing hormone (GnRH) therapy be initiated. The treatment of secondary hypogonadism in women will depend on whether the goal is hormone replacement or ovulation induction. In hormone replacement, estrogen and progestogen preparations, administered orally or through skin patches, can be used. For ovulation induction, gonadotropin therapy should be given for several months, or pulsatile GnRH administration can be used if the deficiency is hypothalamic (AU)

Succinate dehydrogenase D variants do not constitute a risk factor for developing C cell hyperplasia or sporadic medullary thyroid carcinoma.

Medullary thyroid carcinoma (MTC) is a tumor that arises from parafollicular cells of the thyroid gland. MTC can occur sporadically (75%) or as part of inherited cancer syndromes (25%). In most cases, hereditary MTC evolves from preneoplastic C cell hyperplasia (CCH), so early detection of this pathology would evidently be critical. A recent study reports that alterations in succinate dehydrogenase (SDH) D are responsible for familial non-RET CCH. First, we studied SDHD in two families with hereditary non-RET CCH and found no alterations related to the inheritance of this disease. Then, we investigated whether the H50R variant could be a risk factor in the sporadic development of MTC in both Spanish and English patients. We found no evidence that the presence of the H50R is strongly associated with the risk of sporadic MTC, although we did observe an association with age at diagnosis of MTC in Spanish H50R carriers that we did not find in English patients. Finally, we looked for evidence of CCH or any other thyroid disease in a panel of germ-line SDH (B or D) mutation carriers and found none. We conclude that SDHD variants do not constitute a risk factor for developing CCH or sporadic MTC.

Epidemiology, clinical characteristics, outcome, morbidity and mortality in acromegaly based on the Spanish Acromegaly Registry (Registro Espanol de Acromegalia, REA).

OBJECTIVE: To undertake a multicentre epidemiological study reflecting acromegaly in Spain. DESIGN: Voluntary reporting of data on patients with acromegaly to an online database, by the managing physician. METHODS: Data on demographics, diagnosis, estimated date of initial symptoms and diagnosis, pituitary imaging, visual fields, GH and IGF-I concentrations (requested locally), medical, radiotherapy and neurosurgical treatments, morbidity and mortality were collected. RESULTS: Data were included for 1219 patients (60.8% women) with a mean age at diagnosis of 45 years (s.d. 14 years). Reporting was maximal in 1997 (2.1 cases per million inhabitants (c.p.m.) per year); prevalence was globally 36 c.p.m., but varied between 15.7 and 75.8 c.p.m. in different regions. Of 1196 pituitary tumours, most were macroadenomas (73%); 81% of these patients underwent surgery, 45% received radiotherapy and 65% were given medical treatment (somatostatin analogues in 68.3% and dopamine agonists in 31.4%). Cures (GH values (basal or after an oral glucose tolerance test) <2 ng/ml, normal IGF-I, or both) were observed in 40.3% after surgery and 28.2% after radiotherapy. Hypertension (39.1%), diabetes mellitus (37.6%), hypopituitarism (25.7%), goitre (22.4%), carpal tunnel syndrome (18.7%) and sleep apnoea (13.2%) were reported as most frequent morbidities; 6.8% of the patients had cancer (breast in 3.1% of the women and colon in 1.2% of the cohort). Fifty-six patients died at a mean age of 60 years (s.d. 14 years), most commonly of a cardiovascular cause (39.4%); mortality was greater in patients given radiotherapy (hazard ratio 2.29; 95% confidence interval 1.03 to 5.08; P=0.026), and in those in whom GH and IGF-I concentrations were never normal (P<0.001). CONCLUSIONS: This acromegaly registry offers a realistic overview of the epidemiological characteristics, treatment outcome and morbidity of acromegaly in Spain. As active disease and treatment with radiotherapy are associated with an increase in mortality, efforts to control the disease early are desirable.