Feocromocitoma y paraganglioma en un hospital pediátrico de Argentina. Serie de casos / Pheochromocytoma and paraganglioma at a children's hospital in Argentina. A case series

El feocromocitoma y el paraganglioma son tumores neuroendocrinos secretores de catecolaminas. Los feocromocitomas se originan en la médula suprarrenal, mientras que los paragangliomas son extraadrenales. Se describe una serie de casos de niños con diagnóstico anatomopatológico de feocromocitoma o paraganglioma que consultaron en un hospital pediátrico de alta complejidad de Argentina. Se incluyeron 21 pacientes, 14 varones, con una mediana de edad de 11,4 años; 8 casos con feocromocitoma y 13 casos con paraganglioma. Se presentaron con hipertensión arterial 14/21. La mayoría de los paragangliomas tuvieron localización paraaórtica (9/13). Debido a que representan una causa potencialmente curable de hipertensión arterial, la sospecha clínica es muy importante. El diagnóstico temprano y la instauración de un tratamiento antihipertensivo adecuado, que permita afrontar la cirugía con normotensión arterial, aseguran la curación en la mayoría de los casos si la resección tumoral es completa.

Pheochromocytomas and paragangliomas are neuroendocrine tumors producing catecholamines. Pheochromocytomas occur in the adrenal medulla, while paragangliomas are those that occur outside the adrenal gland. Here we describe a case series of children with a pathological diagnosis of pheochromocytoma or paraganglioma who consulted at a tertiary care children's hospital in Argentina. A total of 21 patients (14 males) were included; their median age was 11.4 years; 8 children had pheochromocytoma and 13, paraganglioma. Arterial hypertension was observed in 14/21. Most paragangliomas were para-aortic (9/13). Since they are a potentially curable cause of hypertension, clinical suspicion is very important. An early diagnosis and the initiation of an adequate antihypertensive treatment, which allows the patient to undergo surgery with normal blood pressure, ensure a cure in most cases if tumor resection is complete.

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

Carcinoma adrenal virilizante en una adolescente: reporte de caso / Virilizing adrenal carcinoma in an adolescent: case report

Los tumores suprarrenales virilizante son infrecuentes y representan 5-6% de los tumores de esas glándulas1. Pueden secretar diferentes andrógenos como dehidroepiandrosterona sulfato (DHEAS), androstenediona y testosterona. Las características clínicas dependen de la edad de presentación; en niños pueden determinar pubertad precoz y en mujeres en edad fértil ocasionar hirsutismo, amenorrea o ciclos oligomenorreicos y diversos grados de virilización2. Los carcinomas adrenocorticales son tumores raros y la incidencia es aproximadamente uno a dos por millón de habitantes/año3,4. Los exámenes de imagen como la tomografía o la resonancia confirman el origen suprarrenal, valoran la presencia de metástasis y definen la conducta terapéutica5. La presentación inicial en pacientes pediátricos mayoritariamente es con virilización6 y aproximadamente el 50% de los pacientes adultos con carcinoma adrenal tienen un estadio de la enfermedad relativamente avanzado7. El tratamiento de elección es la cirugía y sigue siendo la mejor esperanza para la supervivencia a largo plazo8. El pronóstico habitual para el carcinoma adrenocortical es generalmente malo con una supervivencia global a 5 años de 20 a 25%5 en adultos, pero en niños y adolescentes la supervivencia puede llegar al 100%9. Se presenta el caso de una paciente con fenotipo totalmente masculino con diagnóstico de carcinoma adrenal virilizante que comienza en la infancia y se diagnostica en la adolescencia.

Virilizing adrenal tumors are uncommon and represent 5-6% on tumors of these glands1. They can secrete different androgens such as dehydroepiandrosterone sulfate (DHEAS), androstenedione, and testosterone. Clinical features depend on the age of presentation; in children they can determine precocious puberty and in women of childbearing age cause hirsutism, amenorrhea or oligomenorrheic cycles and various degrees of virilization2. Diagnosis consists of clinical evidence of hyperandrogenism, accompanied by an increase in androgens in the blood, especially DHEAS, whose origin is mainly adrenal. Adrenocortical carcinomas are rare and the incidence is approximately one to two per million inhabitants/year3,4. Imaging tests such as tomography or resonance confirm the adrenal origin, assess the presence of metastases and define the therapeutic approach5. In initial presentation in most pediatric patients is with virilization6 and approximately 50% adult's patients with adrenal carcinoma have a relatively advanced stage of the disease7. The treatment of choice is surgery and is the best hope for long-term survival. The usual prognosis for adrenocortical carcinoma is generally poor with a 5-year overall survival of 20 to 25%5 in adults, but in children and adolescent's survival can reach 100%9. We present the case of a patient with a totally male phenotype diagnosed with virilizing adrenal carcinoma that begins in childhood and is diagnosed in adolescence.

Presentación atípica de un neuroblastoma congénito metastásico con efecto renal grave: informe de caso / Atypical presentation of a congenital metastatic neuroblastoma with severe renal impact: case report

El neuroblastoma es un tumor derivado de la cresta neural. Se trata del tumor maligno más frecuente en recién nacidos y existe una amplia variabilidad en su forma de presentación. En algunos casos, el diagnóstico se realiza mediante un hallazgo ecográfico, mientras que en otros se manifiesta como enfermedad metastásica agresiva. Se presenta el caso de una paciente prematura con diagnóstico posnatal de neuroblastoma congénito metastásico con manifestaciones clínicas graves y evolución atípica. Asimismo, se destaca una lesión renal grave que requirió terapia dialítica.

Neuroblastoma is a neural crest-derived tumor and the most common malignant tumor in neonates. Its clinical presentation can be quite variable. In some cases, it presents as an aggressive metastatic disease whilst in others it is a finding in scans performed for other reasons. The following report presents the case of a premature newborn, diagnosed postnatally with metastatic congenital neuroblastoma, with an atypical and torpid course, including severe renal injury and dialysis requirement.

Impacto cardiovascular, conducta diagnóstica y terapéutica del feocromocitoma como causa de hipertensión arterial / Cardiovascular impact, diagnostic and therapeutic management of pheochromocytoma as a cause of arterial hypertension

INTRODUCCIÓN. Los feocromocitomas son tumores que provienen de las células neuroendócrinas de la médula adrenal y producen alta secreción de catecolaminas. Generan complicaciones cardiovasculares graves que suelen asociarse con crisis hipertensivas. Es importante valorar el impacto cardiovascular de esta entidad. OBJETIVO. Realizar una revisión exhaustiva de las diversas manifestaciones de los feocromocitomas como causa de hipertensión arterial, su impacto cardiovascular, conducta diagnóstica y terapéutica. MATERIALES Y MÉTODOS. Revisión bibliográfica y análisis de 141 artículos científicos que incluyeron temas sobre el impacto cardiovascular, conducta diagnóstica y terapéutica del feocromocitoma como causa de hipertensión arterial. Se usó bases de datos: Medline, Embase, Scopus, Pubmed, Google Académico. Criterios de búsqueda en DECS, MeSH: "pheochromocytoma OR hypertension arterial AND cardiomyopathy", en inglés- español. Fueron seleccionados: 13 publicaciones de texto completo, 10 artículos retrospectivos, 2 guías de práctica clínica y 1 revisión. Se excluyeron 128 artículos científicos. RESULTADOS. Se realizó una revisión de las manifestaciones clínicas de los feocromocitomas como causa de hipertensión arterial y el impacto cardiovascular se relacionó con la producción de catecolaminas. Para el diagnóstico, la sensibilidad de la resonancia magnética es del 93-100%; la especificidad de resonancia magnética o tomografía computarizada en combinación con gammagrafía con metayodobencilguanidina con 123I es cercana al 100%. La resección del feocromocitoma tiene potencial curativo. CONCLUSIÓN. Los feocromocitomas presentan variabilidad clínica, se asocian a complicaciones cardiovasculares y cerebrovasculares graves por producción de catecolaminas. El diagnóstico oportuno y eficaz debe realizarse mediante resonancia magnética y gammagrafía en caso de alta sospecha clínica. El tratamiento quirúrgico es de elección.

INTRODUCTION. Pheochromocytomas are tumors arising from the neuroendocrine cells of the adrenal medulla and produce high secretion of catecholamines. They generate severe cardiovascular complications that are often associated with hypertensive crises. It is important to assess the cardiovascular impact of this entity. OBJECTIVE. To perform an exhaustive review of the various manifestations of pheochromocytomas as a cause of arterial hypertension, their cardiovascular impact, diagnostic and therapeutic conduct. MATERIALS AND METHODS. Bibliographic review and analysis of 141 scientific articles that included topics on the cardiovascular impact, diagnostic and therapeutic behavior of pheochromocytoma as a cause of arterial hypertension. The following databases were used: Medline, Embase, Scopus, Pubmed, Google Scholar. Search criteria in DECS, MeSH: "pheochromocytoma OR hypertension arterial AND cardiomyopathy", in English-Spanish. The following were selected: 13 full-text publications, 10 retrospective articles, 2 clinical practice guidelines, and 1 review. A total of 128 scientific articles were excluded. RESULTS. A review of the clinical manifestations of pheochromocytoma as a cause of arterial hypertension was performed and the cardiovascular impact was related to catecholamine production. For diagnosis, the sensitivity of MRI is 93-100%; the specificity of MRI or computed tomography in combination with 123I-methiodobenzylguanidine scintigraphy is close to 100%. Resection of pheochromocytoma has curative potential. CONCLUSION. Pheochromocytomas present clinical variability, are associated with severe cardiovascular and cerebrovascular complications due to catecholamine production. Timely and effective diagnosis should be made by MRI and scintigraphy in case of high clinical suspicion. Surgical treatment is the treatment of choice.

Características de incidentaloma suprarrenal durante 10 años en un hospital terciario de Uruguay / Characteristics of adrenal incidentaloma during 10 years in a tertiary hospital in Uruguay

Introducción: la prevalencia de incidentaloma suprarrenal está aumentando por el envejecimiento de la población y el uso generalizado de técnicas de imagen de alta resolución. Objetivo: analizar los casos con incidentaloma suprarrenal en nuestra población. Materiales y métodos: estudio observacional, descriptivo y retrospectivo. Fueron pacientes que se encontraban en seguimiento, desde 2005 al 2015 en Endocrinología del Hospital de Clínicas. En el análisis estadístico se utilizaron medidas de resumen (media, mediana y frecuencias); se realizó análisis bivariados definiendo el punto de significación de 0.05. Resultados: encontramos 42 individuos con incidentaloma suprarrenal. La media de edad: 57.8 años, mayoría mujeres (76.2%). El diagnóstico se realizó por tomografía en 76.2% y el motivo de solicitud fue por causa digestiva en 47.6%. La mediana de tamaño al diagnóstico fue 17 mm, 6 pacientes tuvieron lesiones bilaterales. Se calculó las Unidades Hounsfield a 52.1% de las lesiones y a 18.7% se les realizó washout. Al diagnóstico inicial fueron catalogados 31 pacientes con adenoma no funcionante, 4 con Cushing subclínico, 3 feocromocitomas y 4 quedaron sin catalogar. Se siguieron a los pacientes una mediana de tiempo de 24 meses. De los 4 Cushing subclínicos, 3 se presentaron en lesiones bilaterales, ningún paciente evolucionó a Cushing manifiesto. Crecieron un 18.7% de las lesiones. Se operaron 4 pacientes. Tras la anatomía patológica, uno de los feocromocitomas resultó ser un adenoma. Durante el seguimiento se diagnosticó un feocromocitoma aunque tenía características sospechosas desde el inicio. Ninguno de los bilaterales recibió tratamiento quirúrgico. Conclusiones: se confirmó aumento en el número de casos de incidentaloma suprarrenal del 2005 al 2015. La gran mayoría fueron adenomas no funcionantes. De los Cushing subclínicos tres cuartas partes fueron bilaterales. En la mayoría la conducta fue conservadora.

Introduction: the prevalence of adrenal incidentaloma is increasing due to aging of the population and the widespread use of high-imaging techniques resolution. Objective: to analyze cases with adrenal incidentaloma in our population. Materials and methods: observational, descriptive and retrospective study. They were patients who were in follow-up, from 2005 to 2015 in Endocrinology of the Hospital of Clinics. In the statistical analysis, summary measures were used (mean, median and frequencies); bivariate analyzes were performed defining the significance point of 0.05. Results: we found 42 individuals with adrenal incidentaloma. The average of age: 57.8 years, mostly women (76.2%). The diagnosis was made by tomography in 76.2% and the reason for request was due to digestive causes in 47.6%. The median size at Diagnosis was 17 mm, 6 patients had bilateral lesions. The Units were calculated Hounsfield 52.1% of the injuries and 18.7% had a washout. At the initial diagnosis 31 patients with non-functioning adenoma were classified, 4 with subclinical Cushing, 3 pheochromocytomas and 4 were not cataloged. The patients were followed a median of 24 months' time. Of the 4 subclinical Cushing, 3 were presented in bilateral lesions, no patient evolved to manifest Cushing. They grew 18.7% of the injuries. He 4 patients operated. After the pathological anatomy, one of the pheochromocytomas resulted be an adenoma During the follow-up a pheochromocytoma was diagnosed although suspicious characteristics from the beginning. None of the bilaterals received treatment surgical. Conclusions: an increase in the number of incidentaloma cases was confirmed adrenal from 2005 to 2015. The vast majority were non-functioning adenomas. Of the Cushing subclinical three quarters were bilateral. In the majority, the behavior was conservative.

¿Como optimizar el diagnóstico funcional de los incidentalomas suprrarenales? Importancia de un estudio protocolizado / How to optimize the functional diagnosis of suprarenal incidenta- lomas? Importance of a protocolized study

Adrenal incidentalomas are an increasingly common pathology. Although historically they have been considered largely non-functioning, recent evidence suggests that the usually performed study is incomplete and/or not sensitive enough. In the last decade the clinical spectrum of adrenal hypercortisolism has expanded considerably, including milder cases which are also associated with cardiovascular morbidity and even mortality. Furthermore, primary aldosteronism has also expanded beyond the classic phenotype with advanced vascular damage, resistant hypertension and hypokalemia, currently including asymptomatic, normotensive and normokalemic patients. For this reason, a correct protocolized study is essential in all adrenal incidentalomas, including a precise radiological characterization, as well as a systematic hormonal evaluation using more sensitive cut points. The findings of this workup are relevant, because they allow a more individualized approach to the medical and surgical management of these patients.

Los incidentalomas suprarrenales son una patología cada vez más frecuente. Si bien históricamente han sido considerados no funcionantes en su gran mayoría, evidencia reciente sugiere que el estudio habitual es incompleto y/o poco sensible. En la última década el espectro clínico del hipercortisolismo de origen adrenal se ha ampliado de forma considerable, incluyendo casos leves que también se asocian a morbilidad cardiovascular e incluso mortalidad. Por otro lado, el hiperaldosteronismo primario también ha expandido su fenotipo más allá del clásicamente descrito con daño vascular avanzado, hipertensión resistente e hipokalemia, abarcando en la actualidad a pacientes asintomáticos, normotensos y normokalemicos. Por esta razón es imprescindible un correcto estudio protocolizado en todo incidentaloma suprarrenal, incluyendo una precisa caracterización radiológica, así como una evaluación hormonal sistemática utilizando puntos de corte más sensibles. Los hallazgos de este estudio son relevantes, pues permiten guiar de forma más individualizada el manejo médico y quirúrgico de estos pacientes.

Tumor maligno congénito. Síndrome de Pepper / Congenital malignant tumor: Pepper's Syndrome

Pepper's syndrome refers to a neuroblastoma originated in the adrenal glands that usually metastasizes to the liver with abdominal development and respiratory involvement because of thoracic compression. The metastasic tumors are usually infrequent with an unfavorable prognosis. The cases reported in the world literature are very few. The congenital form of neuroblastoma is uncommon. The aim of this report was to describe a typical clinical case of a new born who died because of a metastasic malignant tumor, comptible with a Pepper's syndrome

Primary malignant tumors of the adrenal glands

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

Feocromocitoma en neoplasia endocrina múltiple IIA asociado a tuberculosis pulmonar presentada como abdomen agudo: reporte de caso y revisión de la literatura / Pheochromocytoma in multiple endocrine neoplasia 2A associated with pulmonary tuberculosis presenting as abdominal pain: a case report and literature review

El feocromocitoma constituye una neoplasia productora de catecolaminas que se presenta de forma esporádica o asociada a enfermedades de transmisión hereditaria, como la neoplasia endocrina múltiple. Los síntomas clásicos como la cefalea, sudoración y palpitaciones son atribuidos a la actividad del sistema nervioso simpático y suelen presentarse en forma de paroxismos. La tuberculosis pulmonar es una enfermedad infecciosa que constituye un problema de salud pública en muchos países, cuya incidencia depende de algunos factores incluyendo la inmunosupresión que generan las enfermedades endocrino-tumorales como la antes descrita. Presentamos el caso de un paciente masculino de 38 años que acude a emergencia por presentar un paroxismo de hipertensión arterial y dolor abdominal, como manifestaciones iniciales de un feocromocitoma en el contexto de una neoplasia endocrina múltiple de tipo IIA. El paciente desarrolló de forma concomitante tuberculosis pulmonar; no obstante, se logró tratar ambas entidades consiguiendo una evolución clínica favorable.

Pheochromocytoma is a catecholamine-producing neoplasm that may occur sporadically or associated with hereditary diseases, such as multiple endocrine neoplasia. The classic symptoms are headache, sweating, and palpitations and are attributed to the sympathetic nervous system activity, usually presenting as paroxysms. On the other hand, pulmonary tuberculosis is an infectious disease considered a public health problem in many countries, whose incidence depends on risk factors such as immunosuppression. It is well known that endocrine-tumor diseases such as multiple endocrine neoplasia can predispose to chronic inflammation and immunosuppression. We report the case of a 38-year-old male patient who had an episode of arterial hypertension and abdominal pain as the first symptoms of a pheochromocytoma associated with multiple endocrine neoplasia type 2A. The patient developed pulmonary tuberculosis simultaneously, but we managed to treat both entities and achieve a favorable clinical course.

Enuresis secundaria como presentación de feocromocitoma en una niña de 13 años / Pheochromocytoma presenting with secondary enuresis in a 13-year-old girl

El feocromocitoma es un tumor raro, infrecuente en la edad pediátrica. Los síntomas clásicos derivados del exceso de catecolaminas son cefalea, sudoración y palpitaciones, aunque los niños pueden tener una clínica más atípica. La hipertensión arterial suele ser un signo constante en la mayoría de los pacientes. Existen pocos casos descritos de poliuria como forma de presentación de feocromocitoma. Se presenta el caso de una niña de 13 años remitida a consulta de Nefrología Pediátrica por enuresis secundaria de un año de evolución. La tensión arterial clínica tomada durante la exploración era superior al percentil 99 para su edad y talla, motivo por el que se decidió el ingreso para su estudio y tratamiento.

Pheochromocytoma is a rare tumor which is infrequent in children. Although the clinical presentation in children can be atypical, the classic symptoms are headache, sweating and tachycardia. Hypertension is often a constant sign in most patients. There are few cases in literature reporting pheochromocytoma presented with polyuria. We present a 13-year-old girl who came to the Pediatric Nephrologist due to a year of evolution of secondary enuresis. When her blood pressure was taken, she was above the 99th percentile that corresponds to her age and her height that is why she was admitted for treatment and diagnostic study.

Miocardiopatía de Tako-Tsubo: una manifestación inusual de feocromocitoma / Takotsubo cardiomyopathy: rare manifestation of pheochromocytoma

Takotsubo cardiomyopathy (MTT) is an acute ventricular dysfunction and reversible in absence of coronary disease. It is a rare presentation of pheochromocytoma and paraganglioma (FPGL). It was described for the first time in 1990 by Sato et al, the physiopathology is not clear yet. It is associated with high levels of catecholamines, vasospasm in the micro vascularization, rupture of atheromatous plaque and myiocarditis. The clinical presentation is similar to an acute myocardial infarction because of that the FPGL must be considered in patients without obstructive coronary lesions. We present a case of a 50 years old women with history of Arterial Hypertension, active smoking and Neurofibromatosis, who is admitted to emergency room with an acute myocardial pain.

Feocromocitoma e gravidez: Relato de caso e revisão atualizada / Pheochromocytoma and pregnancy: A case report and review

Resumo O feocromocitoma é um tumor da adrenal produtor de catecolaminas, sendo causa rara de hipertensão arterial na gravidez. Sua prevalência em hipertensos é de 0,2%, e em 0,002% das gestações. Acompanhamos gestante hipertensa de 24 anos, branca, tercigesta, admitida na 33ª semana com emergência hipertensiva, indicada cesárea devido a sofrimento fetal, evoluindo com edema agudo de pulmão no pós-parto imediato. Submetida à videolaparoscopia após 13 dias por dor abdominal aguda, sem achado significativo. No pós-operatório, devido à hipertensão arterial grave e refratária, suspeitou-se de feocromocitoma, confirmado por exames bioquímicos e de imagem. Realizada adrenelectomia unilateral com cura da hipertensão. A anatomia patológica e a imunohistoquímica confirmaram o diagnóstico. Concluímos que casos atípicos de hipertensão na gravidez devem ser precocemente investigados e diferenciados da pré-eclâmpsia. Apesar da baixa prevalência, o feocromocitoma na gravidez aumenta consideravelmente a morbimortalidade materno-fetal e o seu reconhecimento e tratamento precoces mudam drasticamente o seu desfecho.

Abstract Pheochromocytoma is a catecholamine-producing adrenal tumor, being a rare cause of hypertension in pregnancy. It's prevalence in hypertensive patients is 0.2%, and 0.002% of pregnancies. We follow hypertensive pregnant 24 year old on her third pregnancy, admitted to 33 weeks with hypertensive emergency cesarean section indicated by fetal distress evolving with acute pulmonary edema in the post-partum period. Indicated laparoscopy after 13 days for acute abdominal pain, with no significant finding. In the postoperative, due a severe and resistant hypertension, suspected of pheochromocytoma and confirmed by biochemical tests and imaging. Performed unilateral adrenelectomia with cure of hypertension. The pathology and immunohistochemistry confirmed the diagnosis. We conclude that atypical cases of hypertension in pregnancy should be investigated early and differentiated pre-eclampsia. Despite the low prevalence, pheochromocytoma in pregnancy increases fetal maternal morbidity and mortality and the early recognition and treatment drastically change their outcome.

Factors associated with reporting of abuse against children and adolescents by nurses within Primary Health Care / Fatores associados à notificação de maus-tratos em crianças e adolescentes realizada por enfermeiros na Atenção Primária à Saúde / Factores asociados a la notificación del maltrato en niños y adolescentes realizada por enfermeros en la Atención Primaria a la Salud

OBJECTIVE: to analyze the factors associated with the underreporting on the part of nurses within Primary Health Care of abuse against children and adolescents. METHOD: cross-sectional study with 616 nurses. A questionnaire addressed socio-demographic data, profession, instrumentation and knowledge on the topic, identification and reporting of abuse cases. Bivariate and multivariate logistic regression was used. RESULTS: female nurses, aged between 21 and 32 years old, not married, with five or more years since graduation, with graduate studies, and working for five or more years in PHC predominated. The final regression model showed that factors such as working for five or more years, having a reporting form within the PHC unit, and believing that reporting within Primary Health Care is an advantage, facilitate reporting. CONCLUSION: the study's results may, in addition to sensitizing nurses, support management professionals in establishing strategies intended to produce compliance with reporting as a legal device that ensures the rights of children and adolescents. .

OBJETIVO: analisar os fatores associados à notificação de maus-tratos em crianças e adolescentes, realizada por enfermeiros que atuam na Atenção Primária à Saúde. MÉTODO: estudo transversal, realizado com 616 enfermeiros. Foi utilizado um questionário contendo dados sociodemográficos, formação profissional, instrumentação e conhecimento sobre o tema, identificação e notificação de casos de maus-tratos. Análises bivariada e multivariada por regressão logística foram realizadas. RESULTADOS: predominaram enfermeiros do sexo feminino, na faixa etária entre 21 e 32 anos, não casados, com cinco ou mais anos de formado, com pós-graduação e com cinco ou mais anos de trabalho. O modelo logístico final evidenciou que fatores como tempo de trabalho de cinco ou mais anos, a unidade de saúde possuir a ficha de notificação, saber para onde encaminhar os casos, não ter medo de envolvimento legal e achar vantajosa a notificação na atenção primária facilitam a efetivação do ato notificatório. CONCLUSÃO: os resultados desta pesquisa, além de sensibilizar os enfermeiros para o problema, poderão ser utilizados pelos profissionais da gestão na orientação de estratégias para o cumprimento da notificação como dispositivo legal de garantia dos direitos de crianças e adolescentes. .

OBJETIVO: analizar los factores asociados a la notificación de maltrato en niños y adolescentes realizado por enfermeros que actúan en la Atención Primaria a la Salud. MÉTODO: estudio transversal realizado con 616 enfermeros. Fue utilizado un cuestionario conteniendo datos sociodemográficos, formación profesional, instrumentación y conocimiento sobre el tema, identificación, y notificación de casos de maltrato. Análisis bivariado y multivariado por regresión logística. RESULTADOS: predominaron enfermeros del sexo femenino, en la franja etaria de 21 a 32 años, no casados, con cinco o más años de graduación, con postgraduación y con cinco o más años de trabajo. El modelo logístico final evidenció que factores como tiempo de trabajo de cinco o más años, la unidad de salud poseer ficha de notificación, saber para donde encaminar los casos, no tener miedo de involucramiento legal y encontrar ventaja en la notificación en la atención primaria, son aspectos que facilitan la efectividad del acto de la notificación. CONCLUSIÓN: los resultados de esta investigación, además de sensibilizar a los enfermeros para el problema, podrán ser utilizados por profesionales de la gestión en la orientación de estrategias para el cumplimiento de la notificación como dispositivo legal de garantía de los derechos de niños y adolescentes. .

Diagnóstico, tratamento e seguimento do carcinoma medular de tireoide: recomendações do Departamento de Tireoide da Sociedade Brasileira de Endocrinologia e Metabologia / Diagnosis, treatment, and follow-up of medullary thyroid carcinoma: recommendations by the Thyroid Department of the Brazilian Society of Endocrinology and Metabolism

Introdução O carcinoma medular de tireoide (CMT) origina-se das células parafoliculares da tireoide e corresponde a 3-4% das neoplasias malignas da glândula. Aproximadamente 25% dos casos de CMT são hereditários e decorrentes de mutações ativadoras no proto-oncogene RET (REarranged during Transfection). O CMT é uma neoplasia de curso indolente, com taxas de sobrevida dependentes do estádio tumoral ao diagnóstico. Este artigo descreve diretrizes baseadas em evidências clínicas para o diagnóstico, tratamento e seguimento do CMT. Objetivo O presente consenso, elaborado por especialistas brasileiros e patrocinado pelo Departamento de Tireoide da Sociedade Brasileira de Endocrinologia e Metabologia, visa abordar o diagnóstico, tratamento e seguimento dos pacientes com CMT, de acordo com as evidências mais recentes da literatura. Materiais e métodos: Após estruturação das questões clínicas, foi realizada busca das evidências disponíveis na literatura, inicialmente na base de dados do MedLine-PubMed e posteriormente nas bases Embase e SciELO – Lilacs. A força das evidências, avaliada pelo sistema de classificação de Oxford, foi estabelecida a partir do desenho de estudo utilizado, considerando-se a melhor evidência disponível para cada questão. Resultados Foram definidas 11 questões sobre o diagnóstico, 8 sobre o tratamento cirúrgico e 13 questões abordando o seguimento do CMT, totalizando 32 recomendações. Como um todo, o artigo aborda o diagnóstico clínico e molecular, o tratamento cirúrgico inicial, o manejo pós-operatório e as opções terapêuticas para a doença metastática. Conclusões O diagnóstico de CMT deve ser suspeitado na presença de nódulo tireoidiano e história ...

Introduction Medullary thyroid carcinoma (MTC) originates in the thyroid parafollicular cells and represents 3-4% of the malignant neoplasms that affect this gland. Approximately 25% of these cases are hereditary due to activating mutations in the REarranged during Transfection (RET) proto-oncogene. The course of MTC is indolent, and survival rates depend on the tumor stage at diagnosis. The present article describes clinical evidence-based guidelines for the diagnosis, treatment, and follow-up of MTC. Objective The aim of the consensus described herein, which was elaborated by Brazilian experts and sponsored by the Thyroid Department of the Brazilian Society of Endocrinology and Metabolism, was to discuss the diagnosis, treatment, and follow-up of individuals with MTC in accordance with the latest evidence reported in the literature. Materials and methods: After clinical questions were elaborated, the available literature was initially surveyed for evidence in the MedLine-PubMed database, followed by the Embase and Scientific Electronic Library Online/Latin American and Caribbean Health Science Literature (SciELO/Lilacs) databases. The strength of evidence was assessed according to the Oxford classification of evidence levels, which is based on study design, and the best evidence available for each question was selected. Results Eleven questions corresponded to MTC diagnosis, 8 corresponded to its surgical treatment, and 13 corresponded to follow-up, for a total of 32 recommendations. The present article discusses the clinical and molecular diagnosis, initial surgical treatment, and postoperative management of MTC, as well as the therapeutic options for metastatic disease. Conclusions 7 .

Rare cavernous hemangioma of adrenal gland: case report / Hemangioma cavernoso raro da glândula adrenal: relato de caso

CONTEXT: Cavernous hemangiomas of the adrenal gland are rare benign neoplastic tumors. The clinical presentation of adrenal hemangiomas is usually vague, and they are often discovered incidentally through imaging examination s performed for other reasons. CASE REPORT: We report the case of a non-functional adrenal hemangioma found incidentally in a 37-year-old man with a one-year history of headache and hypertension. A right adrenal mass was detected by means of magnetic resonance imaging. Physical examination and all laboratory values were unremarkable. The patient underwent laparoscopic right adrenal gland resection. Histopathological evaluation confirmed adrenal cavernous hemangioma. CONCLUSIONS: Most occurrences of cavernous hemangiomas of the adrenal gland are non-functional and often discovered incidentally. Although rare, these unusual benign adrenal masses should form part of the differential diagnosis of adrenal neoplasms. The proper treatment for adrenal cavernous hemangioma is surgical removal. .

CONTEXTO: Hemangiomas cavernosos da glândula adrenal são tumores neoplásicos raros, benignos. A apresentação clínica dos hemangiomas adrenais é geralmente vaga e muitas vezes eles são descobertos acidentalmente por exame imagiológico realizado por outras razões. RELATO DE CASO: Nós relatamos um caso de hemangioma adrenal não funcional encontrado por acaso em um homem de 37 anos de idade com histórico de um ano de dor de cabeça e hipertensão. Foi detectada massa adrenal direita por meio de ressonância magnética. Exame físico e todos os valores de laboratoriais estavam normais. O paciente foi submetido a cirurgia laparoscópica para ressecção da glândula adrenal direita. Avaliação histopatológica confirmou hemangioma adrenal cavernoso. CONCLUSÃO: A maioria dos hemangiomas cavernosos de glândula adrenal são não funcionais e muitas vezes descobertos por acaso. Embora raras, essas massas adrenais benignas devem ser parte de um diagnóstico diferencial de neoplasias suprarrenais. O tratamento adequado para o hemangioma adrenal cavernoso é a remoção cirúrgica. .

Descripción de manifestaciones clínico-humorales de feocromocitoma en paciente femenina de 52 años / Clinical and laboratory features of pheochromocytoma in a 52-year-old female patient

El presente artículo busca describir las manifestaciones clínico–humorales ante una paciente con feocromocitoma. Es por ello que se presenta el caso de una paciente de 52 años que acude a la institución por presentar aumento de sus indicadores tensionales acompañado de frialdad de la piel, sudoraciones, nerviosismo, dolor torácico opresivo que aparece varias veces en el día, pérdida de peso en torno a los 13 kilos y decaimiento. Lo más significativo en los estudios realizados fue el ultrasonido abdominal que definió una imagen nodular en la suprarrenal derecha de baja ecogenicidad y contornos regulares que miden 5 mm, confirmada con tomografía axial computarizada contrastada de suprarrenales. Se determinó ácido vanilmandélico hallándose valores aumentados. Asimismo, la biopsia de glándula suprarrenal derecha confirmó la presencia de feocromocitoma de 4,5 x 3,5 x 3 cm.

The article aims to describe the clinical and laboratory features of a female patient suffering from pheochromocytoma. The case is a 52-year-old female patient who presents to our healthcare center with high blood pressure, cold limbs, sweating, jitteriness, and episodes of oppressive chest pain that appear several times per day. She also reports fatigue and a 13-kilogram weight loss. The sonogram revealed a nodular image in the right adrenal gland that had low echogenicity and regular margins measuring 5 mm. The image was confirmed with a contrast-enhanced adrenal CAT scan. Urine vanillylmandelic acid levels were high and an adrenal biopsy confirmed a pheochromocytoma measuring 4.5 x 3.5 x 3 cm.

Feocromocitoma :presentación como síndrome coronario agudo: Reporte de un caso / Presentation of a pheochromocytoma as an acute coronary syndrome: Report of one case

The typical symptoms of pheochromocytoma are palpitations, sweating, headaches and hypertension. We report a 70-year-old female admitted to the hospital due to a sudden onset of precordial pain with electrocardiographic changes. After admission the patient evolved with recurrent chest pain accompanied by hypertensive paroxysms and a pheochromocytoma was suspected. Measurement of catecholamines and metanephrines confirmed the diagnosis and an abdominal magnetic resonance localized the tumor. The patient underwent surgery with successful removal of the pheochromocytoma and was discharged in good conditions.

Computed Tomography and Magnetic Resonance Images of Adrenocortical Oncocytoma Cases

We present two cases of adrenocortical oncocytomas that were well-delineated on multi-detector computed tomography and magnetic resonance imaging. The images showed a well-enhanced large mass with multiple stippled calcifications in a 10-yr-old girl who was consulted due to precocious puberty. A well-enhanced solid mass with necrotic components was incidentally noticed in a 54-yr-old man. These lesions were resected and diagnosed as adrenocortical oncocytomas through immunohistochemical studies and electron microscopy. Adrenocortical oncocytomas are rare disease entities, therefore, we report these interesting, rare adrenocortical oncocytomas here with radiologic findings, and discuss differential diagnosis.

Myxoid Adrenocortical Adenoma: Magnetic Resonance Imaging and Pathology Correlation

We report a case of a 74-year-old female with myxoid adrenocortical adenoma which showed different magnetic resonance imaging findings compared to those of a typical adrenocortical adenoma. The myxoid change in the adrenocortical adenoma is a rare form of degeneration. It presents a considerable diagnostic challenge to both radiologists and clinicians because it can mimic other adrenal tumor types on imaging. The MRI findings of the presented case included a high signal intensity on T2-weighted images similar to that of fluid and delayed progressive enhancement.