Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications

Modifications of adrenal gland ultrastructure in streptozotocin-induced diabetic model rats / Modificaciones de la ultraestructura de la glándula suprarrenal en ratas modelo diabéticas inducidas por estreptozotocina

SUMMARY: The adrenal gland has been associated with the development of classical symptoms in diabetes mellitus (DM), including intensive polyuria and hyperglycemia. During DM, there are hormonal changes in the adrenal gland. Ultrastructural changes of adrenocortical and adrenal medulla cells and their effects on adrenocorticomedullary interaction have not been fully investigated. This study evaluated adrenocortical and adrenal medullary cells and adrenocorticomedullary interactions at ultrastructural levels in a streptozotocin-induced DM model, using transmission electron microscopy. Fifteen male, Sprague-Dawley rats were divided into diabetic model (n = 10) and control (n = 5) groups. Rats were sacrificed at four weeks after induction. The nuclei of some diabetic cortical cells were found to be irregularly shaped. In the cytoplasm, increased numbers of mitochondria and dilated smooth endoplasmic reticulum were observed. However, lipid droplets decreased in the DM model animals. The filopodia of diabetic cortical cells extended to contact the fenestrated capillary and other cortical cells and losses of gap junctions were also observed. Alterations of diabetic chromaffin cells resulted in similar appearances, consisting of irregularly shaped nuclei, swollen mitochondria, distended rough endoplasmic reticulum, and disrupted chromaffin vesicles. Examining adrenocorticomedullary interactions showed that the diabetic cortical chromaffin cells resembled those in the medulla. In the DM model group, collagen fibril depositions were observed between adrenal cells, especially near cell interactions. The filopodia of diabetic cortical cells were larger than those observed for diabetic adrenocorticomedullary interactions and adrenal cortex. These adrenal gland ultrastructural modifications represent contributions to the basic knowledge necessary for investigations of adrenal gland impairment during the early diagnosis of DM patients.

RESUMEN: La glándula suprarrenal se ha asociado con el desarrollo de síntomas clásicos en la diabetes mellitus (DM), que incluyen poliuria intensiva e hiperglucemia. Durante la DM, hay cambios hormonales en la glándula suprarrenal. Los cambios ultraestructurales de las células adrenocorticales y de la médula suprarrenal y sus efectos sobre la interacción adrenocorticomedular no se han investigado completamente. Este estudio evaluó las células adrenocorticales y de la médula suprarrenal y las interacciones adrenocorticomedulares a niveles ultraestructurales en un modelo de DM inducida por estreptozotocina, utilizando microscopía elec- trónica de transmisión. Se dividieron quince ratas macho Sprague- Dawley en grupos de modelo diabético (n = 10) y de control (n = 5). Las ratas se sacrificaron cuatro semanas después de la inducción. Se encontró que los núcleos de algunas células corticales diabéticas tenían una forma irregular. En el citoplasma, se observó un mayor número de mitocondrias y retículo endoplásmico liso dilatado. Sin embargo, las gotitas de lípidos disminuyeron en los animales modelo DM. Los filopodios de las células corticales diabéticas se extendieron para entrar en contacto con el capilar fenestrado y otras células corticales y también se observaron pérdidas de uniones gap. Las alteraciones de las células cromafines diabéticas dieron como resultado apariencias similares, que consistían en núcleos de forma irregular, mitocondrias inflamadas, retículo endoplásmico rugoso distendido y vesículas cromafines rotas. El examen de las interacciones adrenocorticomedulares mostró que las células cromafines corticales diabéticos se parecían a las de la médula. En el grupo del modelo de DM, se observaron depósitos de fibrillas de colágeno entre las células suprarrenales, especialmente cerca de las interacciones celulares. Los filopodios de las células corticales diabéticas eran más grandes que los observados para las interacciones adrenocorticomedulares diabéticas y la corteza suprarrenal. Estas modificaciones ultraestructurales de la glándula suprarrenal contribuyen al conocimiento básico para las investigaciones referente al deterioro de la glándula en el diagnóstico temprano de pacientes con DM.

Giant virilising adrenal cortical carcinoma

Androgen secreting adrenocortical carcinoma (ACC) is a very rare disease with a poor prognosis. Approximately 80% of tumors are functional, most commonly secreting glucocorticoids. We herewith report a case of a huge functional ACC of the right adrenal gland in a 33-year-old female who presented with complaints of hirsutism, amenorrhea and an abdominal lump. On abdominal examination a large lump was palpable in the right hypochondrium reaching up to the umbilicus. Contrast-enhance computed tomography (CECT) revealed a mass in the right suprarenal region. The tumor measured 29 cm × 20 cm × 12 cm and weighed 7.8 kg, the largest reported case of ACC in the world to the best of our knowledge.

Challenging risk factors for right and left laparoscopic adrenalectomy: A single centre experience with 272 cases

ABSTRACT Purpose This study aimed to compare perioperative and postoperative results of right and left laparoscopic adrenalectomy (LA), and to evaluate the impact of challenging factors on these outcomes. Materials and Methods A total of 272 patient's medical records that underwent single side LA between October 2006 and September 2017 were retrospectively reviewed. The patients were divided into 2 groups according to operation side. Moreover, pheochromocytoma, metastatic masses and adrenal lesions >5cm in size were considered to be difficult adrenalectomy cases and the outcomes of these cases were compared between two groups. Results 135 patients (49.6%) underwent right LA and 137 patients (50.4%) underwent left LA. Operation time, estimated blood loss (EBL) and hospitalization time were similar between the groups (p=0.415, p=0.242, p=0.741, respectively). Although EBL was higher on the right side than the left (p=0.038) in the first 20 cases, after this learning period has been completed, there was no significant difference between the groups. In patients with pheochromocytoma, metastatic mass and a mass >5cm in size, despite bleeding complications were clinically higher on the right side, this difference was not statistically significant. Conclusions During the learning period of LA, EBL is higher on the right side. Due to the greater risk of bleeding complications on the right side even on the hands of experienced surgeons, extra care and preoperative planning are required in patients with pheochromocytoma, metastatic masses and masses >5cm in size.

Carcinoma adrenal em crianças: estudo longitudinal em minas gerais, brasil / Adrenal carcinoma in children: longitudinal study in minas gerais, brazil

RESUMO Objetivo: Analisar as características clínicas, laboratoriais e histopatológicas e o percurso até o estabelecimento do diagnóstico e do tratamento de pacientes com carcinoma de suprarrenal (CSR). Métodos: Estudo retrospectivo com 13 pacientes tratados no serviço de oncologia pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais (HC-UFMG) entre 2004 e 2015. Resultados: A idade ao diagnóstico variou de 1,0 a 14,8 anos (mediana: 2,0 anos). As manifestações de hipercortisolismo foram identificadas em todos os casos, e as de virilização, em todas as meninas. Todos os pacientes preencheram os critérios de Weiss para diagnóstico histopatológico de CSR. A imuno-histoquímica foi realizada em 61,5% dos casos. A maioria dos pacientes apresentou doença em estádio I (76,9%). Todos foram submetidos à ressecção tumoral total. Dois pacientes (estádios III e IV) receberam quimioterapia associada ao mitotano. O único óbito observado foi do paciente com doença em estádio IV. A probabilidade de sobrevida global para todo o grupo aos 5,0 anos foi de 92,3±7,4%. A mediana de tempo entre o início dos sintomas e o diagnóstico foi de 9,5 meses, e de 6,0 meses entre a primeira consulta e o início do tratamento. Conclusões: A baixa idade ao diagnóstico, o predomínio de casos com doença localizada e a ressecção tumoral completa - com apenas um caso de ruptura de cápsula tumoral - são possivelmente a explicação para a evolução favorável da população estudada. O longo percurso entre o início dos sintomas e o diagnóstico sugere a importância da capacitação dos pediatras para o reconhecimento precoce dos sinais e dos sintomas do CSR.

ABSTRACT Objective: To analyze clinical, laboratory and histopathological features and the path to diagnosis establishment and treatment of patients with adrenal carcinoma (AC). Methods: Retrospective study with 13 patients assisted at the pediatric oncology service of Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 2004 and 2015. Results: Age at diagnosis ranged from 1.0 to 14.8 years (median: 2.0 years). Manifestations of hypercortisolism were identified in all cases and virilization in all girls. All patients met the Weiss criteria to AC histopathological diagnosis. Immunohistochemistry was performed in 61.5% of the cases. Most patients had stage I disease (76.9%). All subjects were submitted to total tumor resection. Two patients (stages III and IV disease) received chemotherapy associated to mitotane. The only death case was that of a patient with stage IV disease. The probability of overall survival for the entire group up to 5.0 years was 92.3±7.4%. The median time between the onset of symptoms and diagnosis was 9.5 months, and 6.0 months between first visit and start of treatment. Conclusions: Low age at diagnosis, predominance of cases with localized disease and complete tumor resection - with only one case of tumor capsule rupture - can possibly explain the favorable evolution of the studied population. The long period between onset of symptoms and diagnosis highlights the importance of training pediatricians for early recognition of AC signs and symptoms.

Effects of high-fat diets on biochemical profiles and morpho- quantitative characteristics of C57BL/6 mice adrenal glands / Efectos de la dieta alta en grasa sobre el perfil bioquímico y las características morfo-cuantitativas de la glándula suprarrenal de ratón C57BL/6

According to the information provided by the World Health Organization (WHO), overweight and obesity are the fifth leading causes of death in the world. Due to the alarming increase of this disorder in recent years, studies have been carried out to evidence harmful effects on human or animal tissues. Our objective was to evaluate the morphological and physiological changes of C57BL/6 mice adrenal glands (AGs), associated with high fat diets (HFDs), we used 14 male mice, 5 months old, fed for 14 weeks according to two diets. The standard chow group (SC) was fed AIN-93M and the high fat group (HF) was fed AIN-93M-HF. At the end of the experiment, biometric analysis was performed and euthanasia was performed. Blood was then extracted for biochemical analysis and AGs were processed for mesoscopic, histological, morphometric and stereological studies. We used Student's t-test (p<0.05) for statistical analysis. SC group showed a lower weight (29.67±1.28 g) with respect to the HF group (38.46±4.68 g, p=0.002). COL-T, HDL-C, TG and CORT analysis revealed differences between SC group and HF (p≤0.001). Focally, in the fasciculate area, there was an increase in the core-cytoplasm ratio and a greater lipid vacuole presence and size. There was a significant reduction (p=0.001) in Vvfas=7.365±3.326 % of the HF group fasciculate area compared to SC group (Vvfas=9.619±4.548 %). Obesity induced by HF diets affects adrenal gland physiology and morphology of mice. Our results suggest that both the percentage of fat as well as the time of administration of the diet, produce a diurnal reduction of corticosterone, which could be due to an increase in the metabolic clearance of this hormone and not to the inhibition of the Hypothalamus-Pituitary-Adrenal (HPA) axis.

De acuerdo a la información entregada por la OMS, el sobrepeso y la obesidad son el quinto factor principal de riesgo de muerte en el mundo. Debido al aumento alarmante de este trastorno en los últimos años, estudios evidencian efectos perjudiciales sobre tejidos humanos y animales. Por lo tanto, nuestro objetivo fue evaluar los cambios morfo-fisiológicos de la glándula suprarrenal de ratones C57BL/6 asociado a dietas altas en grasas. Utilizamos 14 ratones machos, de 5 meses, alimentados por 14 semanas con dos dietas. Grupo dieta estándar (SC), alimentados con AIN-93M y grupo alto en grasa (HF), alimentado con AIN- 93M-HF. Al finalizar el experimento, se realizó el análisis biométrico y se efectuó la eutanasia. Posteriormente, se extrajo sangre para el análisis bioquímico y cada glándula suprarrenal fue procesada para el estudio mesoscópico, histológico, morfométrico y estereológico. Para el análisis estadístico se utilizó la prueba t de Student (p<0,05). El grupo SC mostró un menor peso (29,67±1,28 g) respecto al grupo HF (38,46±4,68 g; p=0,002). El análisis del COL-T, HDL-C, TG y CORT reveló diferencias entre el grupo SC y HF (p≤0,001). En la zona fasciculada, focalmente, se observó aumento en la relación núcleo-citoplasma y, mayor presencia y tamaño de vacuolas lipídicas. Hubo una reducción significativa (p=0,001) en VVfas=7,365±3,326 % de la zona fasciculada del grupo HF, en comparación al grupo SC (VVfas=9,619±4,548 %). La obesidad inducida por la dieta HF afecta la fisiología y morfología de la glándula suprarrenal de ratones alimentados con dietas altas en grasas. Nuestros resultados sugieren que tanto el porcentaje de grasa, así como el tiempo de administración de la dieta, producen una reducción diurna de la corticosterona, lo que podría ser debido a un aumento en el aclaramiento metabólico de esta hormona y no a la inhibición del eje Hipotálamo-Hipófisis-Adrenal.

B-mode and Doppler ultrasonography of adrenal glands of healthy dogs / Ultrassonografia modo B e Doppler das glândulas adrenais de cães saudáveis

The aim of this study was to determine the vascular indices of adrenal blood flow in healthy dogs (systolic velocity - SV; diastolic velocity - DV; resistance index - RI). Eighteen dogs (thirty six adrenal) were studied. Physical examination, biochemical profile and dexamethasone suppression test were performed to determine general health status. Echotexture, size, contours and margins, and overall shape of the adrenal gland (right and left) were assessed via ultrasound. By spectral Doppler of the phrenic-abdominal artery, the SV, DV, and RI were acquired. Animals did not show alterations in clinical and laboratory examination and suppression of cortisol. Normal homogeneous and echotexture, regular contours and margins and normal shape and size were verified via B mode. Spectral Doppler of the phrenic-abdominal artery showed monophasic-patterned waves and low vascular resistance and systolic peak evident with means values: left adrenal - SV = 31.34cm/s, DV = 9.54cm/s and RI = 0.69; and right adrenal - SV = 27.83cm/s, DV = 7.71cm/s and RI = 0.68. Doppler evaluation of adrenal was easily implemented and may provide base line data in the study, allowing for the use of this technique as a diagnostic tool for diseases of the dog's adrenal.(AU)

Objetivou-se determinar os índices vasculares do fluxo sanguíneo das glândulas adrenais de cães saudáveis (velocidade sistólica - VS; velocidade diastólica - VD; o índice de resistência - IR). Foram utilizados neste estudo 18 cães (36 adrenais). Foram realizados exame físico, perfil bioquímico e teste de supressão com dexametasona para determinar o estado geral de saúde (higidez). Ecotextura, ecogenicidade, tamanho, contornos e margens e o formato das glândulas adrenais (direito e esquerdo) foram avaliados por meio da ultrassonografia convencional. Ao Doppler espectral da artéria frênico-abdominal, foram adquiridos a VS, a VD e o IR. Os animais não apresentaram alterações aos exames clínico, laboratorial e de supressão do cortisol. Ao exame ultrassonográfico, foram verificados ecotextura homogênea, ecogenicidade, formato e tamanhos normais, além de os contornos e as margens serem regulares. Ao Doppler espectral da artéria frênico-abdominal, verificaram-se ondas com padrão monofásico, com resistência vascular baixa e pico sistólico evidente, sendo os valores médios: adrenal esquerda - VS = 31,34cm/s, VD = 9,54cm/s e IR = 0,69; e adrenal direita - VS = 27,83cm/s, VD = 7,71cm/s e IR = 0,68. A avaliação Doppler das adrenais foi facilmente implementada e pode fornecer dados de referência, permitindo a utilização dessa técnica como uma ferramenta de diagnóstico para doenças das adrenais em cães.(AU)

Mielolipoma en una paciente con hiperplasia suprarrenal congénita / Adrenal myelolipoma in a patient with congenital adrenal hyperplasia

Introducción: El mielolipoma es un tumor benigno caracterizado por la presencia de tejido adiposo y elementos de la médula ósea. Se ha visto que puede estar relacionado con niveles elevados de ACTH como en la Hiperplasia Suprarrenal Congénita (HSC). Objetivo: Presentación de un caso clínico. Reporte de caso: Mujer de 64 años de edad con antecedente de Hiperplasia Suprarrenal Congénita en la que se diagnostica de forma incidental un mielolipoma. Conclusiones: Ante lesiones mayores a 5 centímetros, sintomáticas o que sufren algún cambio (clínico o radiológico) durante el seguimiento, se debería considerar el tratamiento quirúrgico con abordaje laparoscópico, el cual sería la mejor opción.

Introduction: Myelolipoma is a benign tumor characterized by the presence of fat and bone marrow elements. We have seen that may be related to elevated levels of ACTH and Congenital Adrenal Hyperplasia (CAH). Objective: Presentation of a case report. Case report: Female 64 years old with a history of congenital adrenal hyperplasia where incidentally diagnosed myelolipoma. Conclusions: In lesions larger than 5 inches, symptomatic or suffering any change (clinical or radiological) during follow-up, should consider surgical treatment with laparoscopic approach, which would be the best option

Adrenal venous sampling in a patient with adrenal Cushing syndrome / Uso del muestreo venoso adrenal en un paciente con un síndrome de Cushing adrenal

The primary bilateral macronodular adrenal hyperplasia or the independent adrenocorticotropic hormone bilateral nodular adrenal hyperplasia is a rare cause hypercortisolism, its diagnosis is challenging and there is no clear way to decide the best therapeutic approach. Adrenal venous sampling is commonly used to distinguish the source of hormonal production in patients with primary hyperaldosteronism. It could be a useful tool in this context because it might provide information to guide the treatment. We report the case of a patient with ACTH independent Cushing syndrome in whom the use of adrenal venous sampling with some modifications radically modified the treatment and allowed the diagnosis of a macronodular adrenal hyperplasia.

La hiperplasia macro nodular bilateral o hiperplasia adrenal nodular bilateral independiente de la hormona adrenocorticotrópica es una de las causas menos frecuentes de hipercortisolismo, su diagnóstico supone un reto y no se tiene claridad de cuál es la mejor aproximación terapéutica. El muestreo venoso adrenal que frecuentemente se utiliza para hacer la distinción del sitio de producción hormonal en el hiperaldosteronismo primario podría ser una herramienta útil en este contexto ya que podría brindar información que pudiera guiar el tratamiento. Presentamos el caso de una paciente con síndrome de Cushing ACTH independiente en quien el uso del muestreo venoso adrenal con algunas modificaciones cambio de manera radical el tratamiento y permitió confirmar una hiperplasia adrenal macro nodular.

Linfoma no Hodgkin suprarrenal primario / Primary Non Hodgkin´s Lymphoma of the Adrenal Gland

El linfoma no Hodgkin primario de la glándula suprarrenal es una patología muy poco común. Su diagnóstico inicial es difícil siendo este histológico. El linfoma B difuso de células grandes es el tipo histológico más frecuente. Se presenta el caso mujer de 62 años a la que se le realiza tomografía axial computarizada abdominal que muestra una masa de 18 cms dependiente de la glándula suprarrenal y con características de carcinoma. Se realiza exéresis de la tumoración, siendo la anatomía patológica linfoma difuso de célula grande B. Este tipo de linfoma tiene mal pronóstico describiéndose supervivencias medias en torno a los 13 meses. No existe un régimen terapéutico bien definido; aunque el tratamiento más aceptado es el esquema R-CHOP. El papel de la radioterapia y de la exéresis tumoral no está claramente establecido. El linfoma suprarrenal primario debe tenerse en cuenta en el diagnóstico diferencial de pacientes que presentan una masa suprarrenal. Ante la sospecha de esta patología es preferible realizar una biopsia con aguja guiada por prueba de imagen para evitar una intervención quirúrgica innecesaria(AU)

Primary non- Hodgkin`s lymphoma of the adrenal gland is very rare. Its initial diagnosis is difficult and needs to be histological. Diffuse large B- cell lymphoma is the most common histological type. This is the report of a 62 years old woman, who was performed abdominal tomography to disclose a 18 cm tumor in the adrenal gland with carcinoma characteristics. The tumor was removed and the final pathologic diagnosis was diffuse large B-cell lymphoma. This type of lymphoma has poor prognosis since the average survival rates are roughly 13 months. There is no well-defined therapeutic regimen, although the most widely accepted treatment is R-CHOP scheme. The roles of radiotherapy and tumor resection are not clearly established. Primary adrenal lymphoma should be considered in the differential diagnosis of patients presenting with an adrenal mass. When this condition is suspected, then it is advisable to perform a imaging-guided needle biopsy to avoid unnecessary surgery(AU)

Optimización del uso de las imágenes en la patología suprarrenal (parte 1) / Optimization of the use of imaging in adrenal pathology (part 1)

Objetivos de aprendizaje: El objetivo de este trabajo es proponer un algoritmo de estudio por imágenes de las lesiones suprarrenales que permita arribar a un diagnóstico correcto en el menor tiempo posible. Esto favorece la adopción de la conducta terapéutica más apropiada para cada patología y puede modificar en forma sustancial un tratamiento. Aunque las características estructurales de las lesiones generalmente son inespecíficas, es posible, a través de las técnicas de imágenes actuales, diferenciar las lesiones compatibles con procesos benignos de las compatibles con procesos malignos y, con la colaboración del endocrinólogo y el laboratorio, también se pueden distinguir las lesiones funcionantes de las no funcionantes. Revisión de tema: Se evaluaron en forma retrospectiva, entre mayo del 2009 y diciembre del 2011, 157 pacientes con patología suprarrenal. En total, eran 95 mujeres y 61 hombres, de 33 a 78 años (media: 55 años). El protocolo de estudio consistió en una tomografía computada (TC) sin contraste intravenoso (IV), con toma de la densidad de la lesión, seguida de una tomografía computada con contraste intravenoso y tiempo de lavado con toma de densidad de la lesión en tiempo portal y tardío a los 15 minutos (tiempo de lavado o wash-out). Las mediciones y cálculos realizados fueron: tamaño de la lesión suprarrenal, densidad media de la misma durante las tres fases estudiadas y porcentajes de lavado relativo y absoluto. Se realizó resonancia magnética (RM) y tomografía computada por emisión de positrones con 18F-fluorodesoxiglucosa (FDG-PET/TC) sólo en aquellos pacientes que así lo requirieron. Además, se compararon los resultados con los de la literatura actualizada sobre el tema. Hallazgos en imágenes: La conjunción de los datos clínicos, de laboratorio y las imágenes con el algoritmo propuesto permitió identificar 59 adenomas no funcionantes (49 unilaterales y 10 bilaterales), 29 adenomas funcionantes, 9 hiperplasias bilaterales, 4...

Objectives of training: The aim of this study is to propose an algorithm for the evaluation of adrenal lesions in order to arrive at the correct diagnosis in the shortest possible time.This method would favor the most appropriate therapeutic procedures for each pathology and may substantially modifi ed a given treatment. In spite of the fact that the structural features of lesions are generally non-specifi c, it is possible with imaging techniques, to establish compatible features which could help us differentiate benign from malignant lesions and with the contribution of the endocrinologist and the laboratory, to distinguish functional from non-functional ones.Topic revision: A retrospective evaluation was carried out from May 2009 to December 2011 on 157 patients with adrenal diseases, including 95 females and 61 males, from 33 to 78 years of age (mean age 55). The study protocol consisted of non-contrasted intravenous computed tomography, with lesion density measurement, followed by contrasted intravenous computed tomography and wash out time with lesion density determination in portal time and later at 15’ during wash out.Measurements and calculations were: adrenal lesion size, medium density and absolute and relative washing percentiles. Magnetic resonance and 18F-fl uorodesoxyglucose positron emission tomography was only performed on those patients who required these techniques. A comparison of the present results with those in the literature is included.Imaging fi ndings: Fifty-nine patients with non-functional adenoma were identifi ed (49 unilateral and 10 bilateral), 29 functional adenomas, 9 with bilateral hyperplasia, 4 myelolipomas (2 in a female patient with simple virilizing congenital adrenal hyperplasia), 2 cysts, 2 hematomas, 3 lymphomas (2 secondary and 1 primary bilateral), 30 metastases, 5 adrenocortical carcinomas, 12 pheochromocytomas,1 malignant pheochromocytoma, and 1 ganglioneuroma...

Disseminated histoplasmosis: A comparative study of the clinical features and outcome among immunocompromised and immunocompetent patients.

Background. Disseminated histoplasmosis is a chronic granulomatous disease caused by the dimorphic fungus, Histoplasma capsulatum. Clinical presentation can vary from the acute pulmonary to the chronic disseminated form. In India, disseminated histoplasmosis often presents with pyrexia of unknown origin with a presentation similar to ‘disseminated tuberculosis’ involving the adrenal glands and bone marrow. Due to rarity of the disease, data are lacking regarding its clinical presentation and outcome among immunocompromised and immunocompetent patients. Methods. During January 2000 to December 2010, we identified 37 patients of disseminated histoplasmosis and attempted to characterize the differences between immunocompromised and immunocompetent patients. Demographic characteristics, clinical presentation, risk factors, laboratory findings, diagnostic yield, treatment received and prognosis were noted and compared between the two groups. Results. Eleven of 37 patients with disseminated histoplasmosis were immunocompromised and 26 were immunocompetent. Comparison of their clinical features showed a higher frequency of skin lesions in the immunocompromised compared to the immunocompetent group (54.5% v. 11.5%). Pancytopenia and anaemia were more common among the immunocompromised (81.8%) compared to the immunocompetent (46.2%) group. In the immunocompromised patients, the diagnosis was made most often by bone marrow aspirate and culture (72.7%) compared to the immunocompromised group where the diagnosis was most often obtained by adrenal gland biopsy and fungal cultures (57.7%). The cure rate was significantly higher in the immunocompetent group (73% v. 45%). Conclusion. The clinical presentation and outcome of patients with disseminated histoplasmosis differs among immunocompromised and immunocompetent patients.

Manejo emergencial do hipoadrenocorticismo em cão – relato de caso / Emergency management of hypoadrenocorticism in dog – case report

O hipoadrenocorticismo é uma endocrinopatia incomum de difícil diagnóstico em cães (ROMÃO e ANTUNES, 2012). Os sinais clínicos são bastante variáveis, e o paciente pode apresentar desde uma simples gastroenterite até sinais de fraqueza e coma, em decorrência da falta de glicocorticóides e mineralocorticóides (ROMÃO e ANTUNES, 2012). O tratamento consiste em restabelecer rapidamente a volemia, principalmente na crise adrenal aguda, com administração de fluidoterapia intravenosa e glicocorticóide (EMANUELLI et al., 2007). O presente trabalho objetiva relatar o tratamento emergencial de um caso de hipoadrenocortiscismo em uma cadela.

Utility of F-18 FDG-PET in Detecting Primary Aldosteronism in Patients with Bilateral Adrenal Incidentalomas

In patients with primary aldosteronism who have bilateral adrenal incidentalomas, it is important to identify which adrenal gland is secreting excess aldosterone. Traditionally, adrenal vein sampling (AVS) has been performed for lateralization despite its invasiveness. Here we report a case of bilateral adrenal incidentaloma in which 18-Fluorodeoxyglucose (FDG)-positron emission tomography (PET) was used to identify the functional adrenal mass. A 53-yr-old man was referred to our clinic due to bilateral adrenal incidentalomas (right: 1 cm, left: 2.5 cm) on computed tomography (CT). Given his history of colon cancer, FDG-PET/CT scanning was used to rule out metastasis. Although there was focal hot uptake lesion in the right adrenal gland, the patient was suspected primary aldosteronism clinically more than metastasis because of the patient's underlying hypertension with hypokalemia. It was consistent with the results of AVS. Based on these findings, we propose that FDG-PET/CT can be used instead of AVS to identify the source of primary aldosteronism between two bilateral adrenal incidentalomas.

MENX and MEN4

Multiple endocrine neoplasias are autosomal dominant disorders characterized by the occurrence of tumors in at least two endocrine glands. Two MEN syndromes have long been known and are well characterized: the MEN type 1 (MEN1) and type 2 (MEN2). These syndromes are caused by germline mutations in the MEN1 and RET genes, respectively, and have a different tumor spectrum. Recently, a variant of the MEN syndromes arose spontaneously in a rat colony and was named MENX. Affected animals consistently develop multiple endocrine tumors, with a spectrum that shares features with both MEN1 and MEN2 human syndromes. Genetic studies identified a germline mutation in the Cdkn1b gene, encoding the p27 cell cycle inhibitor, as the causative mutation for MENX. Capitalizing on these findings, heterozygous germline mutations in the human homologue, CDKN1B, were searched for and identified in patients with multiple endocrine tumors. As a consequence of this discovery, a novel human MEN syndrome, named MEN4, was recognized, which is caused by mutations in p27. Altogether, these studies identified Cdkn1b/CDKN1B as a novel tumor susceptibility gene for multiple endocrine tumors in both rats and humans. Here we review the characteristics of the MENX and MEN4 syndromes and we briefly address the main function of p27 and how they are affected by MENX/4-associated mutations.

unusual hypertensive patient

Many young patients with systemic hypertension have secondary causes which if identified early could lessen the cardiovascular complications associated with long standing hypertension. Coarctation of the aorta is one of those rare causes. We describe a 40 year old male with hypertension, coarctation of the aorta, bicuspid aortic valve, PDA and an adrenal mass

Glucose-dependent insulinotropic peptide receptor overexpression in adrenocortical hyperplasia in MEN1 syndrome without loss of heterozygosity at the 11q13 locus

BACKGROUND: The molecular mechanisms involved in the genesis of the adrenocortical lesions seen in MEN1 syndrome (ACL-MEN1) remain poorly understood; loss of heterozygosity at 11q13 and somatic mutations of MEN1 are not usually found in these lesions. Thus, additional genes must be involved in MEN1 adrenocortical disorders. Overexpression of the glucose-dependent insulinotropic peptide receptor has been shown to promote adrenocortical tumorigenesis in a mice model and has also been associated with ACTH-independent Cushing syndrome in humans. However, to our knowledge, the status of glucose-dependent insulinotropic peptide receptor expression in adrenocortical lesions in MEN1 has not been previously investigated. OBJECTIVE: To evaluate glucose-dependent insulinotropic peptide receptor expression in adrenocortical hyperplasia associated with MEN1 syndrome. MATERIALS/METHODS: Three adrenocortical tissue samples were obtained from patients with previously known MEN1 germline mutations and in whom the presence of a second molecular event (a new MEN1 somatic mutation or an 11q13 loss of heterozygosity) had been excluded. The expression of the glucose-dependent insulinotropic peptide receptor was quantified by qPCR using the DDCT method, and b-actin was used as an endogenous control. RESULTS: The median of glucose-dependent insulinotropic peptide receptor expression in the adrenocortical lesions associated with MEN1 syndrome was 2.6-fold (range 1.2 to 4.8) higher than the normal adrenal controls (p = 0.02). CONCLUSION: The current study represents the first investigation of glucose-dependent insulinotropic peptide receptor expression in adrenocortical lesions without 11q13 loss of heterozygosity in MEN1 syndrome patients. Although we studied a limited number of cases of MEN1 adrenocortical lesions retrospectively, our preliminary data suggest an involvement of glucose-dependent insulinotropic peptide receptor overexpression in the etiology of adrenocortical hyperplasia. New prospective studies will be able to clarify the exact role of the glucose-dependent insulinotropic peptide receptor in the molecular pathogenesis of MEN1 adrenocortical lesions.

Hemorragia suprarrenal espontánea unilateral / Spontaneous unilateral adrenal hemorrhage

Hombre de 73 años que acude al servicio de urgencias por dolor abdominal. Al examen físico se palpa masa que ocupa hipocondrio derecho. Angio TAC muestra masa abdominal de 12 cm dependiente de glándula suprarrenal derecha. Analítica de orina muestra elevación de metanefrinas y normetanefrinas. La gamagrafía fue compatible con feocromocitoma. Se realiza extirpación quirúrgica de masa y la anatomía patológica describe hemorragia suprarrenal sin malignidad.

A 73 year old man, came to the emergency department referring abdominal pain. A palpable mass occupying right upper quadrant on physical examination was identified. CT angiography showed a 12 cm abdominal mass dependent of the right adrenal. Urinalysis showed elevation of metanephrines and normetanephrines. Gamagraphy scan was compatible with pheochromocytoma. Surgical resection of the mass was performed and pathology described an adrenal hemorrhage without malignancy.

Hiperplasia endotelial papilífera de supra-renal: relato de caso / Papillary endothelial hyperplasia of adrenal: case report

Intravascular papillary endothelial hyperplasia is a benign and rare intravascular process thought to arise from an organizing thrombus. Involvement of the adrenal gland is extremely rare, with only one case reported in the literature. We report a case of this vascular lesion in the adrenal gland, treated with laparoscopic adrenalectomy.