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.

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.

Feocromocitoma maligno. A propósito de tres casos / Malignant Pheochromocytoma. As Regards of Three Cases

Introducción: Los feocromocitomas son tumores que se desarrollan a partir de las células cromafines localizadas en la médula suprarrenal, ganglios simpáticos y parasimpáticos. La distinción entre tumores benignos y malignos es compleja, pues no existen marcadores que puedan discriminar esta diferencia con alta sensibilidad y especificidad. Objetivo: Presentar tres casos clínicos de feocromocitoma maligno, diagnosticados y/o tratados en la sala de hospitalización de adultos del INEN. Método: Se revisaron las historias clínicas y se resumieron los datos. Presentación de los casos: Los 3 pacientes eran del sexo masculino, con edades entre 41 y 51 años al diagnóstico del tumor primario. De ellos, dos tenían una hipertensión arterial controlada y el tercero se comenzó a estudiar por crisis paroxísticas de hipertensión; dos tenían antecedentes de diabetes mellitus y no se demostraron evidencias en ningún caso de lesiones en órganos diana por la hipertensión. En los tres pacientes el tumor se localizó en la suprarrenal derecha. Solo en uno de los casos, la etiología maligna fue informada en el estudio anatomopatológico. En los otros dos pacientes, la malignidad se diagnosticó a punto de partida de lesiones metastásicas, años después de la adrenalectomía, en un caso de cadera y en el otro, de pulmón. Conclusiones: El feocromocitoma maligno es una enfermedad infrecuente de la edad mediana y el diagnóstico de su naturaleza es complejo. Se debe sospechar la aparición de enfermedad metastásica ante la reaparición de los síntomas que motivaron el estudio inicial en el paciente, incluso, años después de la adrenalectomía(AU)

Introduction: Pheochromocytomas are tumors developed from chromaffin cells located in the suprarenal medulla, as well as in sympathetic and parasympathetic ganglia. The distinction between benign and malignant tumors is complex, since there are no markers to discriminate this difference with high sensitivity and specificity. Objective: To present three clinical cases of malignant pheochromocytoma, diagnosed and/or treated in the adult hospitalization ward at the National Institute of Endocrinology. Method: The medical records were reviewed and the data was summarized. Case presentation: The three patients were male and aged between 41 and 51 years at the diagnosis of the primary tumor. Of them, two had controlled arterial hypertension and the third began to be studied for paroxysmal hypertension crises; two had a history of diabetes mellitus and no evidence of target-organ lesions due to hypertension was shown in any case. In the three patients, the tumor was located in the right suprarenal gland. Only in one of the cases, malignant etiology was reported in the pathological study. In the other two patients, the malignancy was diagnosed at the starting point of metastatic lesions, years after adrenalectomy: two respective hip and lung cases. Conclusions: Malignant pheochromocytoma is a rare disease that appears at middle age and whose diagnosis is complex, due to its etiological nature. The appearance of metastatic disease should be suspected, given the reappearance of the symptoms that motivated the initial study in the patient, even years after adrenalectomy(AU)

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.

A propósito de un caso de paraganglioma / A propos of a case of paraganglioma

Los paragangliomas son tumores neuroendocrinos que surgen de los paraganglios autonómicos extraadrenales, los cuales son pequeños órganos formados por células derivadas de la cresta neural embrionaria con capacidad de secretar catecolaminas. Los paragangliomas están estrechamente relacionados con los feocromocitomas porque son indistinguibles a nivel celular, y a menudo comparten las mismas manifestaciones clínicas, como hipertensión, cefalea episódica, sudoración y taquicardia. El diagnóstico de estos tumores es importante por su riesgo de malignización, por las implicaciones de otras neoplasias asociadas, y para la posibilidad de realizar estudios genéticos para detección de otros casos dentro de una misma familia. El objetivo de este artículo es desarrollar un resumen sobre la epidemiología, manifestaciones clínicas, pruebas diagnósticas y tratamiento de estos tumores. Se presenta el caso de un joven de raza negra diagnosticado de un paraganglioma(AU)

Paragangliomas are neuroendocrine tumors emerging from the extra-adrenal autonomic paraganglia, which are small organs formed by embryonic neural crest-derived cells with catecholamine-secreting capacity. Paragangliomas are closely linked to pheochromocytomas because they cannot be differentiated at cell level and often share the same clinical manifestations such as hypertension, episodic headache, sweating and tachycardia. The diagnosis of these tumors is important because of risk of becoming malignant, the implications of other related neoplasias and the possibility of making genetic studies to detect other cases in the same family. The objective of this article was to make an abstract about epidemiology, clinical manifestations, diagnostic tests and treatment of these tumors. This is the case of a young Black female who was diagnosed with paraganglioma(AU)

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.

Manejo clínico y quirúrgico de los feocromocitomas y paragangliomas / Clinical and surgical management of pheochromocytomas and paragangliomas

Los feocromocitomas y paragangliomas (Feo/PGL) son tumores neurendocrinos raros con diferentes presentaciones clínicas, asociados a alta morbimortalidad. Reconocer los signos y síntomas es el paso diagnóstico inicial. Las metanefrinas fraccionadas urinarias tienen una excelente sensibilidad y especificidad. La tomografía computarizada (TC) es el método de elección para su localización. La tomografía por emisión de positrones (PET) con F18-fluordeoxiglucosa (F18-FDG) es el método funcional recomendado para detectar metástasis. La resección quirúrgica constituye la única opción curativa en estos pacientes. La adrenalectomía laparoscópica es la vía de abordaje para la mayoría los Feo/PGL. El tratamiento farmacológico, 7 a 14 días previos con alfabloqueantes y betabloqueantes, tiene como objetivo normalizar la presión arterial y prevenir complicaciones cardiovasculares periquirúrgicas. Se conoce que al menos un tercio de los pacientes presentan una mutación genética germinal. El estudio genético debe estar orientado a las características sindrómicas, formas de presentación, localización y fenotipo bioquímico del tumor. Se recomienda el abordaje interdisciplinario en centros especializados con experiencia en esta patología poco frecuente. (AU)

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors with different clinical manifestation associated with high morbidity and mortality. Recognize signs and symptoms is the first step in diagnosis. Urinary fractionated metanephrines have an excellent specificity and sensitivity. Computed Tomography (CT) is the first-choice imaging modality for location. F18-DG positron tomography (PET)/CT scanning is the functional modality of choice for metastatic disease. Surgery is the only curative treatment. Minimally invasive adrenalectomy is the surgical approach for most adrenal pheochromocytomas. Perioperative alpha and beta blockade for 7 to 14 days normalize blood pressure and prevent perioperative cardiovascular complications. Is recognize that at least one-third of the patients have disease-causing germline mutations. Genetic testing must be orientated to syndromic features, presentation, localization and biochemical profile of these tumors. Multidisciplinary teams at centers with appropriate expertise are recommended to ensure a favorable outcome. (AU)

Thunderclap-like headache triggered by micturition and angina as an initial manifestation of bladder pheochromocytoma. A case report / Cefaleia trovoada-símile desencadeada pela micção e angina como manifestação inicial de feocromocitoma de bexiga. Um relato de caso

CONTEXT: Pheochromocytoma is a catecholamine-producing tumor characterized by hypertension, headache, tachycardia, excessive diaphoresis and angina. The thunderclap headache is so named because the pain strikes suddenly and severely. Although the symptoms of bladder pheochromocytoma are rather evident, the diagnosis of this rare neuroendocrine tumor can be missed. CASE REPORT: This study reports the case of a woman diagnosed with bladder pheochromocytoma who experienced thunderclap headache triggered by micturition and angina as an initial manifestation. CONCLUSION: This case study suggests that thunderclap headache and angina occurring concurrently with sudden blood pressure elevation during or immediately after micturition are important diagnostic clues for bladder pheochromocytoma. .

CONTEXTO: Feocromocitoma é um tumor produtor de catecolaminas, caracterizado por hipertensão, cefaleia, taquicardia, sudorese excessiva e angina. A dor de cabeça trovão é assim chamada porque a dor ataca de repente e intensamente. Embora os sintomas de feocromocitoma de bexiga sejam bastante evidentes, o diagnóstico deste tumor neuroendócrino raro pode ser perdido. RELATO DE CASO: Este estudo relata o caso de uma mulher diagnosticada com feocromocitoma na bexiga que sentiu a dor de cabeça trovão, desencadeada pela micção e angina como manifestação inicial. CONCLUSÃO: Este estudo de caso sugere que a dor de cabeça trovão e angina ocorrendo simultaneamente com a elevação da pressão de sangue repentina durante ou imediatamente após a micção são dicas importantes de diagnósticos de feocromocitoma na bexiga. .

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 .

Sinais e sintomas em hipertensão arterial / Signs and symptoms of hypertension

A hipertensão arterial humana primária ou essencial abrange, na sua maioria, indivíduos no estágio I (pressão arterial sistólica [PAS] entre 140 e 160mmHg e pressão arterial diastólica [PAD] entre 90 e 100mmHg) e mesmo em estágios mais avançados (estágio II - PAS até 180mmHg, PAD até 110mmHg). Assintomática por muitos anos, tem sido cunhada como "o matador silencioso". Os sintomas e sinais mais frequentemente observados nos indivíduos hipertensos estão associados à hipertensão arterial de origem secundária ou são consequência da duração e gravidade do comprometimento dos órgãos-alvo (coração, cérebro, rim, olhos e vasos arteriais)...

The primary human arterial hypertension or essential cover mostly individuals in stage I (systolic blood pressure [SBP] between 140 and 160mmHg and diastolic blood pressure [DBP] between 90 and 100mmHg) and even in the most advanced stages (stage II - PAS until 180mmHg, PAD until 110mmHg). Asymptomatic for many years, it has been named as "the silent killer". The symptoms and signals more frequently observed in hypertensive individuals are related to arterial hypertension that has secondary orign or being consequence due duration and gravity of target organ which were affected (heart, brain, kidney, eyes and anterial vessels)...

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.

Extra Adrenal Pheochromocytoma Presenting as Hypertensive Crisis in a Young Male.

Pheochromocytoma is a tumor of aderenal medulla which secrets catecholamines and usually presents as hypertension. Extra-adrenal pheochromocytomas are extremely rare. They occur in the organ of zukerkundle, bladder, retroperitonium, posterior mediastinum and sympathetic chain. We present a case of a young male who presented with hypertension and eventually was found to have extra adrenal pheochromocytoma of the bladder.

Bazo accesorio simulando tumor suprarrenal / Aberrant spleen simulating an adrenal mass

Introduction: The diagnosis of adrenal incidentalomas is common in current clinical practice. Clinical case: We report a 69 years-old female patient with hypertension, who underwent an abdominal CAT Scan, finding a left adrenal mass of 8 cm diameter. Subsequent studies showed elevated urinary metanephrine levels. With the suspicion of a pheochromocytoma, a laparoscopic surgery was performed. The mass resulted to be an aberrant spleen.

Introducción: El diagnóstico de los así llamados "incidentalomas" suprarrenales, cada vez más frecuente en la práctica clínica, plantea un diagnóstico diferencial importante. Caso clínico: Se presenta el caso clínico de una paciente de 69 años, hipertensa, con el hallazgo de una masa suprarrenal izquierda aparentemente funcionante, operada por vía laparoscópica y que resultó ser un bazo aberrante. Se discute el diagnóstico diferencial entre masa suprarrenal y pseudo-tumores adrenales y la embriología y presentación clínica del bazo aberrante.

Composite pheochromocytoma-ganglioneuroma of the adrenal gland: a case report with immunohistochemical study

Composite tumors of the adrenal medulla consisting of pheochromocytoma and ganglioneuroma are rare tumors accounting for less than 3% of all sympathoadrenal tumors. These tumors display more than one line of differentiation in which normal and neoplastic chromaffin cells are capable of differentiating into ganglion cells under the influence of nerve growth factors. To the best of our knowledge, we report the second case with a composite tumor of the adrenal medulla in a normotensive patient from India