ACROSTART: A retrospective study of the time to achieve hormonal control with lanreotide Autogel treatment in Spanish patients with acromegaly / ACROSTART: Estudio retrospectivo del período de tiempo para lograr el control hormonal con lanreótida Autogel en pacientes con acromegalia en la práctica clínica española

Objectives: The ACROSTART study was intended to determine the time to achieve normalization of GH and IGF-I levels in responding patients with acromegaly administered different dosage regimens of lanreotide Autogel (Somatuline(R) Autogel(R)). Methods: From March 2013 to October 2013, clinical data from 57 patients from 17 Spanish hospitals with active acromegaly treated with lanreotide for ≥4 months who achieved hormonal control (GH levels <2.5ng/ml and/or normalized IGF-I levels in ≥2 measurements) were analyzed. The primary objective was to determine the time from start of lanreotide treatment to hormonal normalization. Results: Median patient age was 64 years, 21 patients were male, 39 patients had undergone surgery, and 14 patients had received radiotherapy. Median hormonal values at start of lanreotide treatment were: GH, 2.6ng/ml; IGF-I, 1.6×ULN. The most common starting dose of lanreotide was 120mg (29 patients). The main initial regimens were 60mg/4 weeks (n=13), 90mg/4 weeks (n=6), 120mg/4 weeks (n=13), 120mg/6 weeks (n=6), and 120mg/8 weeks (n=9). An initial treatment regimen with a long interval (≥6 weeks) was administered in 25 patients. Mean duration of lanreotide treatment was 68 months (7-205). Median time to achieve hormonal control was 4.9 months. Injections were managed without healthcare assistance in 13 patients. Median number of visits to endocrinologists until hormonal control was achieved was 3. Fifty-one patients were "satisfied"/"very satisfied" with treatment and 49 patients did not miss any dose. Conclusions: Real-life treatment with lanreotide Autogel resulted in early hormonal control in responding patients, with high treatment adherence and satisfaction despite disparity in starting doses and dosing intervals

Objetivos: El objetivo del estudio ACROSTART era determinar el período de tiempo para lograr la normalización hormonal (GH e IGF-I) en pacientes con acromegalia respondedores al tratamiento considerando los regímenes de lanreótida Autogel (Somatuline(R) Autogel(R)) utilizados en la práctica clínica. Métodos: Desde marzo de 2013 hasta octubre de 2013, en 17 hospitales españoles se analizaron los datos clínicos de 57 pacientes con acromegalia activa tratados con lanreótida durante ≥4 meses que lograron control hormonal (niveles de GH <2,5ng/ml y/o IGF-I normalizado en ≥2 evaluaciones). El objetivo principal fue determinar el período de tiempo desde el inicio del tratamiento con lanreótida hasta la normalización hormonal. Resultados: La mediana de edad de los pacientes fue 64 años, 21 pacientes eran hombres, 39 pacientes habían recibido cirugía, 14 pacientes habían recibido radioterapia. Los valores hormonales medianos al inicio del tratamiento con lanreótida fueron GH: 2,6ng/ml, IGF-I: 1,6×LSN. La dosis inicial más frecuente de lanreótida fue de 120mg (29 pacientes). Los principales regímenes iniciales fueron 60mg/4 semanas (n=13), 90mg/4 semanas (n=6), 120mg/4 semanas (n=13), 120mg/6 semanas (n=6), 120mg/8 semanas (n=9). Se administró un régimen de intervalo prolongado (≥6 semanas) en 25 pacientes. La duración media del tratamiento con lanreótida fue de 68 meses (7-205). El tiempo medio hasta lograr el control hormonal fue de 4,9 meses. Las inyecciones se manejaron sin asistencia médica en 13 pacientes. La mediana del número de visitas al endocrinólogo hasta el control hormonal fue 3. Cincuenta y un pacientes estaban "satisfechos"/"muy satisfechos" con el tratamiento y 49 pacientes no olvidaron ninguna dosis. Conclusiones: El tratamiento en la vida real con lanreótida Autogel condujo a un control hormonal temprano en pacientes que respondieron, con una alta adherencia al tratamiento y satisfacción con el tratamiento, a pesar de la disparidad de las dosis iniciales y los intervalos de dosificación

ACROSTART: A retrospective study of the time to achieve hormonal control with lanreotide Autogel treatment in Spanish patients with acromegaly.

OBJECTIVES: The ACROSTART study was intended to determine the time to achieve normalization of GH and IGF-I levels in responding patients with acromegaly administered different dosage regimens of lanreotide Autogel (Somatuline® Autogel®). METHODS: From March 2013 to October 2013, clinical data from 57 patients from 17 Spanish hospitals with active acromegaly treated with lanreotide for ≥4 months who achieved hormonal control (GH levels <2.5ng/ml and/or normalized IGF-I levels in ≥2 measurements) were analyzed. The primary objective was to determine the time from start of lanreotide treatment to hormonal normalization. RESULTS: Median patient age was 64 years, 21 patients were male, 39 patients had undergone surgery, and 14 patients had received radiotherapy. Median hormonal values at start of lanreotide treatment were: GH, 2.6ng/ml; IGF-I, 1.6×ULN. The most common starting dose of lanreotide was 120mg (29 patients). The main initial regimens were 60mg/4 weeks (n=13), 90mg/4 weeks (n=6), 120mg/4 weeks (n=13), 120mg/6 weeks (n=6), and 120mg/8 weeks (n=9). An initial treatment regimen with a long interval (≥6 weeks) was administered in 25 patients. Mean duration of lanreotide treatment was 68 months (7-205). Median time to achieve hormonal control was 4.9 months. Injections were managed without healthcare assistance in 13 patients. Median number of visits to endocrinologists until hormonal control was achieved was 3. Fifty-one patients were "satisfied"/"very satisfied" with treatment and 49 patients did not miss any dose. CONCLUSIONS: Real-life treatment with lanreotide Autogel resulted in early hormonal control in responding patients, with high treatment adherence and satisfaction despite disparity in starting doses and dosing intervals.

Linfoma esplénico primario productor de vitamina D: una causa excepcional de hipercalcemia / Vitamin D producer primary splenic lymphoma: an exceptional cause of hypercalcemia

No disponible

[Anthropometric parameters in evaluating malnutrition in oncological patients; utility of body mass index and percentage of weight loss]. / Parámetros antropométricos en la evaluación de la malnutrición en pacientes oncológicos hospitalizados; utilidad del índice de masa corporal y del porcentaje de pérdida de peso.

OBJECTIVE: To compare the BMI and the percentage of weight loss as markers for malnutrition in hospitalized cancer patients considering the Patient-Generated Global Subjective Assessment (PG-GSA) as the gold standard. METHOD: Cross-sectional descriptive study in patients admitted to the Medical Oncology Department of the Hospital Xeral de Vigo, from May to September of 2011. RESULTS: 28 patients (15 males). Mean age 63.46 years ± 11.05. Mean BMI 23.75 kg/m² ± 3.62. Mean percentage of weight loss 8.53% ± 6.20. In group A (well nourished) the percentage of weight loss was 1.07 ± 1.85, in group B (moderately malnourished) 7.90 ± 1.73, and in group C (severely malnourished) 10.91 ± 6.91 (p = 0.034). The BMI showed no statistically significant differences. CONCLUSIONS: The BMI is not a proper parameter todetect malnutrition, by contrast with the percentage of weight loss that did show a direct association with the degree of hyponutrition.

Objetivo: Comparar el IMC y el porcentaje de pérdida de peso como marcadores de malnutrición en el paciente oncológico hospitalizado tomando como referencia la Valoración Subjetiva Global Generada por el Paciente (VSG-GP). Método: Estudio descriptivo transversal en pacientes ingresados en Oncología Médica del Hospital Xeral de Vigo de mayo a septiembre de 2011. Resultados: 28 pacientes (15 varones). Edad media 63,46 años ± 11,05. IMC medio 23,75 kg/m2 ± 3,62. Porcentaje medio de pérdida de peso 8,53% ± 6,20. En el grupo A (bien nutridos) el porcentaje de pérdida de peso fue de 1,07 ± 1,85, en el B (moderadamente desnutridos) de 7,90 ± 1,73 y en el C (severamente desnutridos) 10,91 ± 6,91 (p = 0,034). El IMC no obtuvo diferencias estadísticamente significativas. Conclusiones: El IMC no es un parámetro adecuado para detectar malnutrición a diferencia del porcentaje de pérdida de peso que sí mostró una asociación directa con el grado de desnutrición.

Parámetros antropométricos en la evaluación de la malnutriciónen pacientes oncológicos hospitalizados; utilidad del índice de masacorporal y del porcentaje de pérdida de peso / Anthropometry parameters in evaluating malnutrition in oncological patients; utility of body mass index and percentage of weight loss

Objetivo: Comparar el IMC y el porcentaje de pérdida de peso como marcadores de malnutrición en el paciente oncológico hospitalizado tomando como referencia la Valoración Subjetiva Global Generada por el Paciente (VSG-GP). Método: Estudio descriptivo transversal en pacientes ingresados en Oncología Médica del Hospital Xeral de Vigo de mayo a septiembre de 2011. Resultados: 28 pacientes (15 varones). Edad media 63,46 años ± 11,05. IMC medio 23,75 kg/m2 ± 3,62. Porcentaje medio de pérdida de peso 8,53% ± 6,20. En el grupo A (bien nutridos) el porcentaje de pérdida de peso fue de 1,07 ± 1,85, en el B (moderadamente desnutridos) de 7,90 ± 1,73 y en el C (severamente desnutridos) 10,91 ± 6,91 (p = 0,034). El IMC no obtuvo diferencias estadísticamente significativas. Conclusiones: El IMC no es un parámetro adecuado para detectar malnutrición a diferencia del porcentaje de pérdida de peso que sí mostró una asociación directa con el grado de desnutrición (AU)

OBJECTIVE: To compare the BMI and the percentage of weight loss as markers for malnutrition in hospitalized cancer patients considering the Patient-Generated Global Subjective Assessment (PG-GSA) as the gold standard. METHOD: Cross-sectional descriptive study in patients admitted to the Medical Oncology Department of the Hospital Xeral de Vigo, from May to September of 2011.RESULTS:28 patients (15 males). Mean age 63.46 years ± 11.05. Mean BMI 23.75 kg/m² ± 3.62. Mean percentage of weight loss 8.53% ± 6.20. In group A (well nourished) the percentage of weight loss was 1.07 ± 1.85, in group B (moderately malnourished) 7.90 ± 1.73, and in group C (severely malnourished) 10.91 ± 6.91 (p = 0.034). The BMI showed no statistically significant differences. CONCLUSIONS: The BMI is not a proper parameter to detect malnutrition, by contrast with the percentage of weight loss that did show a direct association with the degree of hyponutrition (AU)

The use of ultrasound elastography in the assessment of malignancy risk in thyroid nodules and multinodular goitres.

OBJECTIVE: The objective of our work is to use a surgical series to analyse the validity of elastography in evaluating the malignancy of thyroid nodules and multinodular goitres. PATIENTS AND METHODS: The study examined 156 patients, 134 women (85·9%) and 22 men (14·1%), who underwent surgery for nodular thyroid disease between October 2008, and November 2010. The average age of the patients was 52 years (±14·42). Thirty-six patients (23·1%) presented with a single nodule, and 120 (76·9%) exhibited multinodular goitres. Prior to surgical intervention, all patients underwent colour eco-elastography using a qualitative five-point ordinal scale for nodule classifications. Test results were analysed using a receiver operating characteristic (ROC) curve, and an anatomical pathologic examination of surgical specimens was used as a gold standard diagnostic tool for comparison. RESULTS: Of the 192 nodules analysed, 28 (14·6%) were malignant. Using elastography data, an ROC curve was obtained with an area under the curve of 0·662 (±0·060) and a 95% confidence interval (CI 95%) between 0·545 and 0·779 (P = 0·006). By establishing a cut-off point that classified thyroid nodules with an elastographic value greater than or equal to 3 as malignant, we achieved a sensitivity of 75%, specificity of 45·73%, positive predictive value of 19·1% and negative predictive value of 91%. The positive and negative likelihood ratios were 1·38 and 0·55, respectively. CONCLUSIONS: Ultrasound elastography can provide information regarding malignancy risk in thyroid nodules and multinodular goitres. However, the sensitivity and specificity values obtained in our study are below those reported by other groups, a finding that calls into question the current clinical utility of ultrasound elastography.

[Phenotype of the C634Y mutation in the RET proto-oncogene in MEN2A: report of a family]. / Fenotipo de la mutación C634Y del protooncogén RET en el MEN2A: a propósito de una familia.

BACKGROUND AND OBJECTIVES: Genetic testing of RET proto-oncogen allows an early diagnosis of Multiple Endocrine Neoplasia syndrome type 2 and establish a correlation between genotype and clinical manifestations. The purpose of this study was to demonstrate the benefits of an early diagnosis with genetic testing followed by prompt surgery on the cure of MTC versus a later diagnosis with serum calcitonin. PATIENTS AND METHOD: Retrospective descriptive study of 8 members of a MEN 2A family by C634Y mutation. We performed serum calcitonin screening until 1999 and subsequently RET genetic testing was obtained. Carriers underwent total thyroidectomy and periodic determination of calcitonin, urinary metanephrines, calcium, phosphorus and neck and abdominal imaging techniques. RESULTS: Five patients were diagnosed by calcitonin familial screening and all of them have high calcitonin by now. Three patients were diagnosed by genetic testing (an adult and two children) and they are free of disease. Calcitonin was closely monitored in children and they underwent surgery when it started to raise, at 6 and 10 years old respectively, finding nodular C-cell hyperplasia in both. Of 8 carriers 3 developed pheochromocytomas, bilateral and asynchronous, one-half had normal urinary metanephrines and two of them were simultaneous with MTC. No patient had biochemical data suggesting hyperparathyroidism although in one patient multiple parathyroid adenomas were found at thyroidectomy. CONCLUSIONS: RET genetic analysis has achieved an early diagnosis and treatment with no development of MTC in our patients, adjusting the time and type of surgery and allowing a genotype-phenotype correlation. It demonstrates how a genetic alteration is associated with a pathology that we can prevent and manage improving the prognosis of our patients.

Fenotipo de la mutación C634Y del protooncogén RET en el MEN2A: a propósito de una familia / Phenotype of the C634Y Mutation in the RET Proto-oncogene in MEN2A: Report of a Family

Antecedentes y objetivos: El estudio genético del protooncogén RET permite un diagnóstico precoz del síndrome de neoplasia endocrina múltiple tipo 2 y establece una correlación entre el genotipo y las manifestaciones clínicas. El objetivo del presente trabajo es demostrar los beneficios del diagnóstico precoz por estudio genético seguido de tratamiento temprano en la curación del carcinoma medular de tiroides (CMT) frente al diagnóstico más tardío con la calcitonina sérica. Pacientes y método: Estudio descriptivo retrospectivo de 8 miembros de una familia con MEN2A por mutación C634Y. Se realizó despistaje con calcitonina sérica hasta 1999 y estudio genético de RET posteriormente. A los portadores se les realizó tiroidectomía total y determinaciones periódicas de calcitonina, metanefrinas urinarias, calcio, fósforo y pruebas de imagen a nivelcervical y abdominal. Resultados: Los 5 pacientes diagnosticados por despistaje familiar con calcitonina presentan en la actualidad cifras de calcitonina elevadas. Los 3 diagnosticados por estudio genético (un adulto y dos niños) se encuentran libres de enfermedad. En los niños se monitorizó la calcitonina y se les intervino cuando esta comenzó a elevarse, a los 6 y 10 años respectivamente, hallándose hiperplasia nodular de células C en ambos. De los 8 afectos 3 presentaron feocromocitomas, bilaterales y asincrónicos, la mitad con metanefrinas urinarias (..) (AU)

Background and objectives: Genetic testing of RET proto-oncogen allows an early diagnosis of Multiple Endocrine Neoplasia syndrome type 2 and establish a correlation between genotype and clinical manifestations. The purpose of this study was to demonstrate the benefits of a nearly diagnosis with genetic testing followed by prompt surgery on the cure of MTC versus alater diagnosis with serum calcitonin. Patients and method: Retrospective descriptive study of 8 members of a MEN 2A family byC634Y mutation. We performed serum calcitonin screening until 1999 and subsequently RET genetic testing was obtained. Carriers underwent total thyroidectomy and periodic determination of calcitonin, urinary metanephrines, calcium, phosphorus and neck and abdominal imagingte chniques. Results: Five patients were diagnosed by calcitonin familial screening and all of them have highcalcitonin by now. Three patients were diagnosed by genetic testing (an adult and two children)and they are free of disease. Calcitonin was closely monitored in children and they underwentsurgery when it started to raise, at 6 and 10 years old respectively, finding nodular C-cellhyperplasia in both. Of 8 carriers 3 developed pheochromocytomas, bilateral and asynchronous,one-half had normal urinary metanephrines and two of them were simultaneous with MTC. Nopatient had biochemical data suggesting hyperparathyroidism although in one patient multipleparathyroid adenomas were found at thyroidectomy. Conclusions: RET genetic analysis has achieved an early diagnosis and treatment with nodevelopment of MTC in our patients, adjusting the time and type of surgery and allowing agenotype-phenotype correlation. It demonstrates how a genetic alteration is associated with apathology that we can prevent and manage improving the prognosis of our patients (AU)

Técnicas de radiodiagnóstico en tumores neuroendocrinos gastroenteropancreáticos / Radiodiagnostic techniques in gastroenteropathic neuroendocrine tumors

Los avances tecnológicos en tomografía computarizada y resonancia magnética han permitido que estas técnicas jueguen un papel cada vez más importante en el manejo de los pacientes con tumores neuroendocrinos que, por otro lado, siguen siendo un reto incluso para radiólogos con gran experiencia en estas neoplasias fascinantes, como demuestran las diferentes series publicadas. El desarrollo de la tomografía computarizada multidetector ha mejorado la sensibilidad en la detección de los tumores de islotes pancreáticos, así como los elevados gradientes y otros avances en resonancia magnética. Ambas técnicas parecen tener una sensibilidad similar pero la comparación es difícil entre las distintas modalidades de imagen y los diferentes modos o estadios del desarrollo tumoral. En tumores carcinoides, tanto la tomografía computarizada como la resonancia magnética son importantes a la hora de definir la extensión de la enfermedad metastásica y evaluar la respuesta al tratamiento. Ambas técnicas, junto con la ecografía endoscópica, pueden ser críticas a la hora de localizar la fuente de un tumor pancreático endocrino funcionante. Los resultados están en relación directa con la experiencia y el interés del radiólogo, la meticulosidad en la técnica y la resolución de los distintos métodos de imagen. Sin embargo, la mayoría de las veces se necesita una combinación de varias pruebas de localización anatómica con estudios de localización funcional y con radioisótopos para encontrar estas escurridizas lesiones (AU)

Because of the technical advances made in computed tomography (CT) and magnetic resonance imaging (MRI), these techniques have become increasingly important in the management of patients with neuroendocrine tumors which, as shown by several published series, continue to pose a challenge even for radiologists highly experienced in these fascinating neoplasms. The development of multidetector CT, as well as high-gradient MRI and other advances in this modality, have improved the sensitivity of pancreatic islet tumor detection. The sensitivity of the two techniques seems to be similar, but comparison between the distinct imaging modalities and the different modes or stages of tumoral development is difficult. In carcinoid tumors, both CT and MRI are important to define the extension of metastatic disease and to evaluate treatment response. Both techniques, together with endoscopic ultrasonography, can be essential when identifying the source of a functioning pancreatic endocrine tumor. The results are directly related to the radiologist’s interest and experience, the meticulousness with which the technique is performed, and the resolution of the distinct imaging methods. However, a combination of various techniques including functional localization and radioisotope use is usually required to detect these elusive lesions (AU)

[Behçet disease with isolated ACTH deficiency]. / Enfermedad de Behçet asociada a déficit de corticotropina.

We report a case of a patient with longstanding Behçet disease, with neurological symptoms predominantly, who became hospitalized for adrenal insufficiency, caused by isolated deficiency of corticotropin (DAACTH). DAACTH is a typical characteristic of hypophysitis, reported in association with many autoimmune diseases. Nevertheless, hypothalamic-pituitary injury in Behçet disease is exceptional. We review the literature and possible mechanisms of this association until now not reported.

Cromogranina A. La familia cromogranina-secretogranina / Chromogranin-A. The chromogranin-secretogranin family

Las cromograninas A y B, y la secretogranina II constituyen los principales miembros de una familia de proteínas solubles, las graninas, localizadas en la matriz de los gránulos secretorios de la mayoría de las células neuroendocrinas, y que incluyen también a las menos conocidas secretograninas III-VII. Aunque genéticamente diferentes, se caracterizan por compartir almacenamiento y secreción con otras hormonas y péptidos en las células del sistema neuroendocrino difuso, participar activamente en el proceso de formación del gránulo secretorio y presentar numerosos puntos para su procesamiento proteolítico por las enzimas PC1/3 y PC2, coalmacenadas también en el gránulo secretorio, y dar lugar a péptidos con propiedades reguladoras, funcionando así comoprohormonas. Las graninas, en general, y la cromogranina A, en particular, tendrían, así,3 funciones principales: a) papel intracelular crucial en la formación de los gránulos de secreción y en la liberación de hormonas y neurotransmisores en las células neuroendocrinas; b) comoprohormonas, a nivel extracelular, siendo precursores de varios péptidosbioactivos derivado de su procesamiento proteolítico, actuando de forma autocrina, paracrina y endocrina, y c) papel como marcador no específico,concretamente la cromogranina A, en el manejo de neoplasiasneuroendocrinas (AU)

Chromogranin A (CgA), B (CgB) and secretogranin II (SgII) are the principal members of a family of soluble proteins called granins, which are found in thematrix of the secretory granules of the majority of neuroendocrine cells, and which also include the lesser known secretogranins III - VII. Although genetically different, they are characterized by sharing storage and secretion with other hormones and peptides in the cells of the diffuse neuroendocrine system, actively participating in secretory granule formation, with numerous sites for the proteolytic enzymes PC1/3 and PC2, which are also stored in the secretory granule, and producing peptides with regulatory properties, thus functioning as prohormones. Therefore, granins in general, and CgA in particular, should have three main functions: a crucial intracellular role insecretory granule formation and hormone and neurotransmitter release in the neuroendocrine cells: b) as pro-hormones, at extracellular level, due to being the precursors of several bioactive peptides produced by their proteolytic process, having autocrine, paracrine and endocrine functions; and c) a role as a non-specificmarker, particularly chromogranin A, in the management of neuroendocrine neoplasias (AU)

Similitudes y discrepancias en las características clinicopatológicas de feocromocitomas y paragangliomas / Clinical and pathological similarities and differences between pheochromocytomas and paragangliomas

Objetivo: Comparar características clínicas, principalmente adrenérgicas, de los feocromocitomas (FC), paragangliomas simpáticos (PGLS) y parasimpáticos (PGLPS).Pacientes y método: Se incluyó en el estudio a 34 pacientes diagnosticados y tratados consecutivamente de FC (20), PGLS (9) y PGLPS (5), con una edad media de 44 (rango, 17-74) años. Se comparó formas de presentación, manifestaciones clínicas, producción hormonal, características patológicas y resultados tras el tratamiento. Resultados: Los pacientes fueron atendidos por múltiples servicios. Los FC se diagnosticaron predominantemente por clínica adrenérgica (65%); los PGLS, por clínica adrenérgica (44%) y por efecto compresivo (45%), y los PGLPS, por efecto masa (100%). Sin embargo, cuando se evaluó de forma dirigida la clínica adrenérgica, el 80% de los FC, el 66,7% de los PGLS y el 40% de los PGLPS la habían manifestado. Presentaron secreción hormonal el 82,4% de los FC y el 100% de los PGLS. No se realizó estudio bioquímico en 3 FC, 5 PGLS y en ningún PGLPS. El tamaño tumoral (en mm) de los FC fue significativamente mayor que los PGLS y PGLPS (59 ± 28, 39 ±16 y 34 ± 26). Tras la extirpación del tumor, 17pacientes con FC, 7 con PGS y 4 con PGLPS quedaron libres de enfermedad, en 1 con PGLPS, éste persiste parcialmente, 1 PGLS recidivó y 5 pacientes murieron (3 con FC y 2 con PGLS).Conclusiones: En nuestro estudio observamos que se diagnostica por clínica adrenérgica los FC (65%) y los PGLS (44%), y además los PGLPS también presentaron clínica compatible con producción de catecolaminasen el interrogatorio dirigido en un 40%. Queremos reseñar el alto porcentaje de manifestaciones adrenérgicas en el grupo de tumores extra adrenales, que no se suele estudiarlos en este sentido, por tanto creemos que deben ser manejados como una misma entidad nosológica por una unidad de referencia (AU)

Objective: To compare the clinical features, mainly adrenergic manifestations, in patients with pheochromocytoma (PCT), sympathetic paraganglioma (SPGL) and parasympathetic paraganglioma (PSPGL).Patients and method: Thirty-four patients consecutively diagnosed and treated for PCT (n = 20), SPGL (n = 9) and PSPGL (n = 5) were included in the present study. The mean age was 44 years (range, 17-74). The forms of presentation, clinical manifestations, results of diagnostic tests, pathological characteristics, and postsurgery outcomes were analyzed. Results: The patients were treated in different medical units in our hospital. Diagnosis of PCT was mainly based on adrenergic symptoms (65%), SPGL on adrenergic symptoms (44%) and compressive symptoms (65%) and PSPGL on the basis of the tumor mass effect (100%). However, exhaustive analysis of adrenergic manifestations revealed that 80%, 66.7% and 40% of patients with PCT,SPGL and PSPGL respectively had adrenergic manifestations. Eighty-two percent of patients with PCT and 100% of those with SPGL showed increased urinaryexcretion of catecholamines and their metabolites. Biochemical studies were not performed in three patients with PCT and five with SPGL and were performed in all patients with PSPGL. The mean tumoral size (in mm) was significantly higher in PCT than in SPGL and PSPGL (59 ± 28, 39 ± 16 and 34 ± 26). After surgery, 17 patients with PCT, seven with PGS and four with PSPGL were considered cured, one patient showed partially persistent PSPGL, one patient with SPGL relapsed, and five patients died (three with PCT and two with SPGL).Conclusions: In the present study, 65% of PCT and 44% of SPGL were diagnosed on the basis of adrenergic symptoms, while 40% of PSPGL also showed symptoms compatible with catecholamine production. We highlight the high percentage of adrenergic manifestations in the group of extra-adrenal tumors, which are not usually investigated in this context. Therefore, we believe that PCT, SPGL and PSPGL should be considered as the same nosological entity in different locations and should be managed in referral units (AU)

Hiperparatiroidismo, bocio y carcinoma tiroideo bien diferenciado: una relación frecuente con implicaciones diagnósticas / Hyperparathyroidism, goiter and well-differentiated thyroid carcinoma. A common association with diagnostic implications

Objetivo. Estudiar la enfermedad tiroidea encontrada en pacientes intervenidos por hiperparatiroidismo (HPT) comparando la frecuencia de carcinomas tiroideos bien diferenciados entre los distintos tipos de HPT. Pacientes y método. Revisión retrospectiva de las historias clínicas de 124 pacientes intervenidos de HPT de manera consecutiva en nuestro servicio por un mismo cirujano entre enero de 1998 y junio de 2006. Resultados. Se trata de 105 (84,7%) mujeres y 19 (15,3%) varones, con una media de edad de 59,7 ± 15,66 años. De ellos, 115 (92,7%) presentan HPT primario (HPTP) y 9 (7,3%), HPT renal (HPTR). En 3 (2,6%) HPTP y en ninguno de los HPTR hay antecedente de radioterapia cervical. En 43 (34,7%) pacientes se realizó simultáneamente algún tipo de cirugía del tiroides, 40 (34,8%) de los HPTP y 3 (33,3%) de los HPTR (p = 1,00). Entre los 115 pacientes intervenidos por HPTP, encontramos 6 (5,2%) carcinomas papilares y entre los 9 afectos de HPTR, 2 (22,2%), sin diferencias estadísticamente significativas entre ambos grupos (p = 0,105). Conclusiones. La afección tiroidea es frecuente en pacientes intervenidos por HPT, pero no más que en series de autopsias. No se encuentran diferencias significativas en la prevalencia de carcinoma papilar de tiroides entre los HPT primario y renal. La radioterapia cervical no parece ser en la actualidad un factor clínicamente relevante en el desarrollo de carcinoma papilar de tiroides en los pacientes afectos de HPTP. La prevalencia de enfermedad tiroidea en la población general justifica la realización de una ecografía preoperatoria de forma sistemática (AU)

Objective. To analyze thyroid gland disease incidentally found in patients undergoing surgery for hyperparathyroidism and to compare the frequency of nonmedullary thyroid carcinoma among different hyperparathyroidism varieties. Patients and method. We retrospectively reviewed 124 clinical records of patients who underwent surgery for hyperparathyroidism by a single surgeon from January 1998 to June 2006. Results. There were 105 women (84.7%) and 19 men (15.3%). The mean age was 59.7 ± 15.66 years old. Of these, 115 (92.7%) were diagnosed with primary hyperparathyroidism and 9 (7.3%) with renal hyperparathyroidism. In the primary hyperparathyroidism group, 3 patients (2.6%) had received neck irradiation compared with none in the renal hyperparathyroidism group. Thyroid gland surgery was performed simultaneously in 43 patients (34.7%), 40 (34.8%) of them belonging to the first group and three (33.3%) to the second group (p = 1.00). Among the 115 patients operated on for primary hyperparathyroidism, 6 papillary thyroid carcinomas (5.2%) were found and 2 (22.2%) were found among the 9 patients with renal hyperparathyroidism, but this difference was not statistically significant (p = 0.105). Conclusions. An incidental finding of thyroid disease in patients undergoing surgery for hyperparathyroidism is fairly frequent but no more so than that found in autopsy series. No statistically significant differences were found between primary and renal hyperparathyroidism in the prevalence of papillary thyroid carcinoma. Currently, neck irradiation does not seem to be a clinically significant factor in this issue. The high prevalence of thyroid disease in the general population warrants systematic neck ultrasound before hyperparathyroidism surgery (AU)

[Hyperparathyroidism, goiter and well-differentiated thyroid carcinoma. A common association with diagnostic implications]. / Hiperparatiroidismo, bocio y carcinoma tiroideo bien diferenciado: una relación frecuente con implicaciones diagnósticas.

OBJECTIVE: To analyze thyroid gland disease incidentally found in patients undergoing surgery for hyperparathyroidism and to compare the frequency of nonmedullary thyroid carcinoma among different hyperparathyroidism varieties. PATIENTS AND METHOD: We retrospectively reviewed 124 clinical records of patients who underwent surgery for hyperparathyroidism by a single surgeon from January 1998 to June 2006. RESULTS: There were 105 women (84.7%) and 19 men (15.3%). The mean age was 59.7 +/- 15.66 years old. Of these, 115 (92.7%) were diagnosed with primary hyperparathyroidism and 9 (7.3%) with renal hyperparathyroidism. In the primary hyperparathyroidism group, 3 patients (2.6%) had received neck irradiation compared with none in the renal hyperparathyroidism group. Thyroid gland surgery was performed simultaneously in 43 patients (34.7%), 40 (34.8%) of them belonging to the first group and three (33.3%) to the second group (p = 1.00). Among the 115 patients operated on for primary hyperparathyroidism, 6 papillary thyroid carcinomas (5.2%) were found and 2 (22.2%) were found among the 9 patients with renal hyperparathyroidism, but this difference was not statistically significant (p = 0.105). CONCLUSIONS: An incidental finding of thyroid disease in patients undergoing surgery for hyperparathyroidism is fairly frequent but no more so than that found in autopsy series. No statistically significant differences were found between primary and renal hyperparathyroidism in the prevalence of papillary thyroid carcinoma. Currently, neck irradiation does not seem to be a clinically significant factor in this issue. The high prevalence of thyroid disease in the general population warrants systematic neck ultrasound before hyperparathyroidism surgery.

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)