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Diabetes & Metabolism - In Press.Proof corrected by the author Available online since samedi 23 decembre 2017

Background Neuroendocrine tumors (NETs) are an unusual family of neoplasms with a wide and complex spectrum of clinical behavior. Here, we present the first report of a National Cancer Registry of gastroenteropancreatic neuroendocrine tumors from a Southern European country. Patients and methods Data was provided online at www.retegep.net by participating centers and assessed for internal consistency by external independent reviewers. Results The study cohort comprised 907 tumors. The most common tumor types were carcinoids (55%), pancreatic nonfunctional tumors (20%), metastatic NETs of unknown primary (9%), insulinomas (8%) and gastrinomas (4%). Forty-four percent presented with distant disease at diagnosis, most often those from small intestine (65%), colon (48%), rectum (40%) and pancreas (38%), being most unusual in appendix primaries (1.3%). Stage at diagnosis varied significantly according to sex, localization of primary tumor, tumor type and grade. Overall 5-year survival was 75.4% (95% confidence interval 71.3% to 79.5%) and was significantly greater in women, younger patients and patients with hormonal syndrome and early stage or lower grade tumors. Prognosis also differed according to tumor type and primary tumor site. However, stage and Ki-67 index were the only independent predictors for survival. Conclusion This national database reveals relevant information regarding epidemiology, current clinical practices and prognosis of NETs in Spain, providing valuable insights that may contribute to understand regional disparities in the incidence, patterns of care and survival of this heterogeneous disease across different continents and countries.

El Grupo de Trabajo de Neuroendocrinologia de la Sociedad Espanola de Endocrinologia y Nutricion (SEEN) ha desarrollado y potenciado en los ultimos anos diferentes subgrupos. El Registro Espanol de Acromegalia (REA) ha continuado su labor incrementando los registros, lo que ha permitido aumentar los datos de morbilidad y mortalidad de esta enfermedad. Igualmente, en el presente ano se ha procedido a la creacion de una nueva aplicacion online con mejoras, tanto en su aspecto formal como de actualizacion de los datos recogidos. El Registro Espanol de Tumores Gastroenteropancreaticos (RETEGEP), en el que participan diferentes especialidades medicas, ha experimentado un aumento significativo de registros, aunque desde la endocrinologia el numero de nuevos casos se ha estabilizado. Por otra parte, se ha llevado a cabo la creacion del Grupo Espanol de Tumores Neuroendocrinos, bajo los auspicios de la Sociedad Espanola de Oncologia Medica y la propia SEEN. El Registro de Neoplasias Endocrinas Multiples tipo 1, por el contrario, se halla en una trayectoria dificil, que obliga a una reflexion de su futuro. El subgrupo de trabajo del sindrome de Cushing, que tambien pertenece al Grupo de Trabajo de Neuroendocrinologia, ha tomado en el ultimo ano nuevo impulso, con estructuracion tanto de su composicion como de sus objetivos. Por ultimo, el Grupo de Trabajo de Neuroendocrinologia de la SEEN ha desarrollado, en el presente ano, la elaboracion de guias clinicas del diagnostico y el tratamiento del prolactinoma y de otros estados de hiperprolactinemia, de la acromegalia y de la deficiencia de hormona de crecimiento del adulto, que vieron la luz en el primer numero de esta revista del ano 2005. Se estan ultimando las guias clinicas de 3 nuevas enfermedades, y se estan en proyecto otras tantas para el curso proximo. El grupo se halla abierto al apoyo y la colaboracion de todos los socios de la SEEN.

Concepto. Cuadro clinico derivado de la exposicion cronica y aumentada a los glucocorticoides. Etiopatogenia. La ACTH plasmatica permite categorizar al sindrome de Cushing en dependiente, bien de origen hipofisario o ectopico, o bien independiente de origen primario suprarrenal. Manifestaciones clinicas. Sindrome de Cushing y sindrome metabolico. Muchos de los signos y sintomas del sindrome de Cushing son muy frecuentes en la poblacion general. Ello implica que con frecuencia se plantee el diagnostico del sindrome y se deba realizar el despistaje de este. Diagnostico. El diagnostico del sindrome de Cushing obliga a la aplicacion de diversos pasos de forma gradual. Tras el despistaje y el diagnostico sindromico, se realizan las pruebas para determinar la etiologia del sindrome. Con relativa frecuencia las pruebas no son concluyentes y esto obliga a efectuar pruebas mas especificas. Tecnicas de imagen. La resonancia es la tecnica de imagen de eleccion en la caracterizacion morfologica del sindrome de Cushing de origen hipofisario. La tomografia axial computarizada es la exploracion de imagen para la visualizacion de las glandulas adrenales. Tratamiento del sindrome de Cushing. La cirugia hipofisaria es la tecnica de eleccion en el sindrome de Cushing de origen hipofisario. Sin embargo, el cuadro presenta con frecuencia situaciones de persistencia o recidiva de la enfermedad. La patologia adrenal primaria tiene en la mayor parte de los casos una indicacion de cirugia adrenal. Se revisa, asimismo, el papel de la radioterapia hipofisaria, y el tratamiento medico.

With puromycin aminonucleoside-induced nephrotic syndrome (NS) in rats, twofold elevated levels of lipoproteins were observed. These levels were not related to proteinuria or to plasma albumin levels. Ultrastructural lesions induced in the kidneys by puromycin aminonucleoside were consistent with NS, while there was little or no hepatic involvement. Apolipoprotein B (apo B) kinetic measurements using homologous 125I-labeled low density lipoproteins (LDL) demonstrated a higher synthetic rate in nephrotic rats relative to controls (6.18 +/- 1.86 micrograms x g-1 x d-1 v 3.94 +/- 0.66 micrograms x g-1 x d-1 respectively, P less than .005), while the fractional catabolic rate was only marginally reduced (1.64 +/- 0.28 pools x day-1 in NS v 1.83 +/- 0.37 pools x day-1 in controls, P less than 0.4). These results indicate that in rats with experimentally induced NS, the expanded apo B-LDL pool results from increased synthesis of this apoprotein while no significant role can be ascribed to alterations in its catabolism. These data are consistent with our preliminary findings in NS in humans.

Serum gonadal hormones, gonadotrophins and zinc levels were studied in thirteen men aged 29-62 yr with chronic renal failure undergoing haemodialysis. All patients had decreased libido and impotence. Serum testosterone levels in patients (18.5 +/- 1.3 (SEM) nmol/l) were significantly lower (p less than 0.05) than in the control group (24.1 +/- 2.2 (SEM) nmol/l) although salivary testosterone levels were strictly within the normal range. Mean serum 17-beta-oestradiol and luteinizing hormone levels (0.19 +/- 0.03 (SEM) nmol/l, and 57.4 +/- 13.1 (SEM) IU/l, respectively) were significantly higher (p less than 0.05 and p less than 0.005, respectively) than in the control group (0.11 +/- 0.02 (SEM) nmol/l and 14.8 +/- 1.9 (SEM) IU/l, respectively). Mean progesterone and follicle-stimulating hormone levels in patients were not significantly different from those of control subjects. Mean prolactin values in patients (1,019 +/- 285 (SEM) mIU/l) were significantly higher (p less than 0.01) than in the control group (211 +/- 24 (SEM) mIU/l). Serum prolactin levels in five patients were extremely high (above 1,200 mIU/l). There was no statistically significant difference in serum zinc levels between patients and controls. As salivary testosterone is normal, it seems that hyperprolactinaemia and raised serum 17-beta-oestradiol levels may be responsible, at least in part, for sexual dysfunction in male patients with chronic renal failure receiving haemodialysis.