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.

Cefalea e hipertensión como forma de presentación de un feocromocitoma y simulando un síndrome post-COVID-19 / Headache and hypertension as a form of presentation of pheochromocytoma and simulating a post-COVID-19 syndrome

El síndrome post-COVID-19 es un conjunto de síntomas y signos que persisten durante más de 12 semanas después de una infección por COVID-19 y actualmente carece de una definición clínica estandarizada. Únicamente se ha informado un caso en el que un feocromocitoma se confundió con un síndrome post-COVID-19. La sintomatología de este síndrome es variable y abarca desde la cefalea y la fatiga hasta la disnea persistente y las alteraciones neurocognitivas. Además, el SARS-CoV-2 puede afectar al sistema nervioso autónomo, contribuyendo a síntomas que se asemejan a los del feocromocitoma. Se recalca la importancia y la necesidad de discernir entre síntomas relacionados con la COVID-19 y otras afecciones, ya que la especificidad de las manifestaciones clínicas del síndrome post-COVID-19 es muy baja y puede ser confundido con otras enfermedades vitales. Se presenta un caso en el que un feocromocitoma fue confundido con un síndrome post-COVID-19 en una paciente sin antecedentes médicos.(AU)

Post-COVID-19 syndrome is a set of symptoms and signs that persist for more than 12 weeks after COVID-19 infection and currently lacks a standardised clinical definition. Only one case has been reported in which a pheochromocytoma was mistaken for post-COVID-19 syndrome. The symptomatology of this syndrome is variable and ranges from headache and fatigue to persistent dyspnoea and neurocognitive disturbances. In addition, SARS-CoV-2 can affect the autonomic nervous system, contributing to symptoms resembling those of pheochromocytoma. The importance and need to discern between COVID-19-related symptoms and other conditions is emphasised, as the specificity of the clinical manifestations of post-COVID-19 syndrome is very low and can be confused with other vital pathologies. A case is presented in which a pheochromocytoma was mistaken for post-COVID-19 syndrome in a patient with no medical history.(AU)

[Headache and hypertension as a form of presentation of pheochromocytoma and simulating a post-COVID-19 syndrome]. / Cefalea e hipertensión como forma de presentación de un feocromocitoma y simulando un síndrome post-COVID-19.

Post-COVID-19 syndrome is a set of symptoms and signs that persist for more than 12 weeks after COVID-19 infection and currently lacks a standardised clinical definition. Only one case has been reported in which a pheochromocytoma was mistaken for post-COVID-19 syndrome. The symptomatology of this syndrome is variable and ranges from headache and fatigue to persistent dyspnoea and neurocognitive disturbances. In addition, SARS-CoV-2 can affect the autonomic nervous system, contributing to symptoms resembling those of pheochromocytoma. The importance and need to discern between COVID-19-related symptoms and other conditions is emphasised, as the specificity of the clinical manifestations of post-COVID-19 syndrome is very low and can be confused with other vital pathologies. A case is presented in which a pheochromocytoma was mistaken for post-COVID-19 syndrome in a patient with no medical history.

Cardiopatía isquémica como forma de presentación de feocromocitoma / Ischemic cardiomyopathy as a presentation of pheocromocytoma

El feocromocitoma es una causa poco frecuente de cardiopatía isquémica. Presentamos el caso de una paciente en la que se diagnostica de un feocromocitoma a raíz de una cardiopatía isquémica con ausencia de lesiones coronarias, demostrando la importancia de tenerlo en cuenta en el diagnóstico diferencial en estos casos, sobre todo teniendo en cuenta que disponemos de un tratamiento curativo. (AU)

Pheochromocytoma is a rare cause of ischaemic heart disease. We present the case of a patient in whom pheochromocytoma was diagnosed following ischaemic heart disease with absence of coronary lesions, demonstrating the importance of taking it into account in the differential diagnosis in these cases, especially considering that curative treatment is available. (AU)

Evaluación del incidentaloma adrenal / Evaluation of the adrenal incidentaloma

An adrenal incidentaloma is a mass detected by chance on an imaging test. It is important that general practitioners know how to manage it, since it is an increasingly frequent finding. It is necessary to assess whether it presents malignant characteristics in imaging tests and the possibility of subclinical hormonal hyperfunction. The basic hormonal study evaluates the overproduction of catecholamines with a study of fractionated metanephrines in plasma or in 24-hour urine, hypercortisolism with a dexamethasone suppression test, and, in patients with hypertension or hypokalemia, the possibility of hyperaldosteronism with determination of the plasma aldosterone/renin ratio. Surgical treatment is indicated in hyperfunctioning nodules, those with suspicion of malignancy or in large ones. (AU)

El incidentaloma adrenal es una masa detectada de forma casual en una prueba de imagen. Es importante que los médicos generalistas conozcan su manejo, ya que es un hallazgo cada vez más frecuente. Hay que valorar si presenta características de malignidad en las pruebas de imagen y la posibilidad de hiperfuncionalidad hormonal subclínica.El estudio hormonal básico evalúa la sobreproducción de catecolaminas con un estudio de metanefrinas fraccionadas en plasma o en orina de 24 horas, el hipercortisolismo con una prueba de frenación con dexametasona y, en pacientes con hipertensión o hipopotasemia, la posibilidad de hiperaldosteronismo con la determinación del cociente aldosterona/renina plasmáticas. El tratamiento quirúrgico está indicado en nódulos hiperfuncionantes, con sospecha de malignidad o en aquellos de gran tamaño. (AU)

[Ischemic cardiomyopathy as a presentation of pheocromocytoma]. / Cardiopatía isquémica como forma de presentación de feocromocitoma.

Pheochromocytoma is a rare cause of ischaemic heart disease. We present the case of a patient in whom pheochromocytoma was diagnosed following ischaemic heart disease with absence of coronary lesions, demonstrating the importance of taking it into account in the differential diagnosis in these cases, especially considering that curative treatment is available.

Histogram analysis in the differentiation between adrenal adenomas and pheochromocytomas: the value of a single measurement / Análise por histograma para diferenciação entre adenomas da adrenal e feocromocitomas: o valor de uma medida única

Abstract Objective: To assess the diagnostic accuracy of histogram analysis on unenhanced computed tomography (CT) for differentiating between adrenal adenomas and pheochromocytomas (PCCs). Materials and Methods: We retrospectively identified patients with proven PCCs who had undergone CT examinations between January 2009 and July 2019 at one of two institutions. For each PCC, we selected one or two adenomas diagnosed within two weeks of the date of diagnosis of the PCC. For each lesion, two readers scored the size, determined the mean attenuation, and generated a voxel histogram. The 10th percentile (P10) was obtained from the conventional histogram analysis, as well as being calculated with the following formula: P10 = mean attenuation - (1.282 × standard deviation). The mean attenuation threshold, histogram analysis (observed) P10, and calculated P10 (calcP10) were compared in terms of their diagnostic accuracy. Results: We included 52 adenomas and 29 PCCs. The sensitivity, specificity, and accuracy of the mean attenuation threshold were 75.0%, 100.0%, and 82.5%, respectively, for reader 1, whereas they were 71.5%, 100.0%, and 81.5%, respectively, for reader 2. The sensitivity, specificity, and accuracy of the observed P10 and calcP10 were equal for both readers: 90.4%, 96.5%, and 92.6%, respectively, for reader 1; and 92.3%, 93.1%, and 92.6%, respectively, for reader 2. The increase in sensitivity was significant for both readers (p = 0.009 and p = 0.005, respectively). Conclusion: For differentiating between adenomas and PCCs, the histogram analysis (observed P10 and calcP10) appears to outperform the mean attenuation threshold as a diagnostic criterion.

Resumo Objetivo: Avaliar a acurácia diagnóstica da análise por histograma na tomografia computadorizada (TC) sem contraste para a diferenciação entre adenomas adrenais e feocromocitomas (FCCs). Materiais e Métodos: Identificamos, retrospectivamente, pacientes com diagnóstico de FCC confirmado que foram submetidos a exames de TC entre janeiro de 2009 e julho de 2019 em duas instituições distintas. Para cada FCC, selecionamos um ou dois adenomas diagnosticados em até duas semanas da data do diagnóstico do FCC. Para cada lesão, dois leitores pontuaram o tamanho, determinaram a atenuação média e geraram um histograma com os voxels das imagens. O percentil 10 (P10) foi obtido a partir da análise convencional do histograma, além de ser calculado com a seguinte fórmula: P10 = atenuação média - (1,282 × desvio-padrão). O limiar de atenuação média, o P10 da análise por histograma (P10 observado) e o P10 calculado (P10calc) foram comparados em termos de acurácia diagnóstica. Resultados: Foram incluídos 52 adenomas e 29 FCCs. A sensibilidade, especificidade e acurácia do limiar de atenuação média foram de 75,0%, 100,0% e 82,5% para o leitor 1, respectivamente, e de 71,5%, 100,0% e 81,5% para o leitor 2, respectivamente. A sensibilidade, especificidade e acurácia do P10 observado e do P10calc foram idênticas para os dois leitores: 90,4%, 96,5% e 92,6%, respectivamente, para o leitor 1; e 92,3%, 93,1% e 92,6%, respectivamente, para o leitor 2. O aumento da sensibilidade foi significativo para ambos os leitores (p = 0,009 e p = 0,005, respectivamente). Conclusão: Para a diferenciação entre adenomas e FCCs, a análise por histograma (P10 observado ou P10calc) parece superar o limiar de atenuação média como critério diagnóstico.

Guía colombiana de práctica clínica para el diagnóstico y tratamiento del incidentaloma adrenal / Colombian clinical practice guideline for diagnostic and treatment of adrenal incidentaloma

Objetivo: Brindar recomendaciones actualizadas a urólogos y profesionales de la salud involucrados en el diagnóstico y manejo del incidentaloma adrenal. Métodos: Mediante la adopción de la guía para manejo de incidentaloma adrenal de la Sociedad Europea de Endocrinología (AGREE-II y AGREE-REX) y búsqueda complementaria de literatura basada en la mejor evidencia científica disponible en definición, diagnóstico, manejo quirúrgico y seguimiento. Adicionalmente, esta guía aborda pacientes con incidentalomas bilaterales y embarazadas. Resultados: Incidentaloma adrenal se define como una lesión mayor de 1 cm localizado en la suprarrenal, detectada mediante una imagen realizada por una razón diferente a cualquier sospecha de patología adrenal. La gran mayoría son adenomas no funcionantes, que no representan riesgo y no requieren manejo adicional. Sin embargo, existen lesiones tumorales como el carcinoma adrenocortical, el feocromocitoma, adenomas productores de hormonas o metástasis. Conclusiones: Los incidentalomas adrenales son masas predominantemente benignas que no requieren adrenalectomía, no obstante se requiere estudiarlas para descartar patologías que requieran manejo específico. Enfáticamente, la intervención quirúrgica debe guiarse por la probabilidad de malignidad, grado de secreción hormonal, edad, estado de salud y preferencia del paciente.

Objective: To provide updated recommendations to urologists and health-care providers faced to diagnosis and treatment of adrenal incidentaloma. Methods: Through adoption of the adrenal incidentaloma guideline from European Endocrinology Society (AGREE-II and AGREE-REX), and complementary search of literature based on available high-quality scientific evidence for definition, diagnosis, surgical management and follow-up. Additionally, this guideline covers bilateral adrenal incidentalomas and pregnant women. Results: Adrenal incidentaloma is defined as a lesion greater than 1 cm localized in adrenal gland, detected by imaging studies which are requested for another different reason than an adrenal pathology suspicion. A great majority are non-functional adenomas, without life-threatening risk nor additional treatment necessity. However, there are tumoral lesions that demand appropriate management like adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma, or metastasis. Conclusions: Adrenal incidentalomas predominantly are benign masses that do not require adrenalectomy (specially in asymptomatic, unilateral, non-functioning adrenal mass), nevertheless, must be exclude other harmful pathologies. Emphatically, surgical treatment must be indicated by malignant probability, hormonal-secreting status, age, health condition, and patient's preference.

Manifestaciones endocrinológicas en la enfermedad de Von Hippel-Lindau: revisión narrativa

La enfermedad de Von Hippel-Lindau es un síndrome neoplásico, autosómico dominante, caracterizado por una mutación germinal del gen VHL que codifica para la proteína VHL en el cromosoma 3. Esta mutación predispone al desarrollo de tumores benignos y malignos que afectan diferentes órganos, a causa de una ausencia de la inhibición de la vía de la tumo-rigénesis mediada por el factor inducible por hipoxia. La prevalencia de esta enfermedad es de 2 a 3 por 100 000 personas y las neoplasias se localizan con mayor frecuencia en retina, sistema nervioso central, cabeza y cuello, páncreas, riñón, glándula suprarrenal y órgano reproductor. Se clasifica en 2 tipos dependiendo de la presencia o ausencia de feocromocitoma. El feocromocitoma y las neoplasias pancreáticas constituyen las manifestaciones endocrinas más frecuentes. El feocromocitoma se presenta entre el 10-30% de los casos. Puede cursar desde una entidad asintomática hasta una sintomatología variable que incluye la triada clásica de cefalea, palpitaciones y diaforesis. El diagnóstico se realiza mediante pruebas bioquímicas o sus metabolitos que confirman niveles elevados de catecolaminas, y estudios imagenológicos. Las lesiones pancreáticas son con frecuencia asintomáticas y se detectan de forma incidental en estudios de imagen realizados en los pacientes con VHL. Aunque las características clínicas y bioquímicas de estas neoplasias no son patognomóni-cas, pueden ser útiles para sugerir la enfermedad VHL como la etiología subyacente.

Von Hippel-Lindau disease is an autosomal dominant neoplastic syndrome characterized by a germline mutation of the VHL gene encoding the VHL protein on chromosome 3. This mutation predisposes to the development of benign and malignant tumors that affect different organs, due to an absence of inhibition of the hypoxia-inducible factor-mediated tumorigenesis pathway. The prevalence of this disease is 2 to 3 per 100,000 people, and neoplasms are most frequently located in the retina, central nervous system, head and neck, pancreas, kidney, adrenal gland, and the organ. It is classified into 2 types depending on the presence or absence of pheochromocytoma. Pheochromocytoma and pancreatic neoplasms are the most frequent endocrine manifestations. Pheochromocytoma occurs in 1030% of cases. It can range from an asymptomatic entity to a variable symptomatology that includes the classic triad of headache, palpitations and diaphoresis. The diagnosis is made through biochemical tests that confirm high levels of catecholamines and imaging studies. Pancreatic lesions are frequently asymptomatic and are detected incidentally in imaging studies performed in VHL patients. Although the clinical and biochemical characteristics of these malignancies are not pathognomonic, they may be useful in suggesting VHL disease as the underlying etiology.

Role of imaging test with radionuclides in the diagnosis and treatment of pheochromocytomas and paragangliomas.

Radionuclide imaging tests with [123I] Metaiodobenzylguanidine (MIBG), [18F] -fluorodeoxyglucose, [18F]-fluorodopa, or 68Ga-DOTA(0)-Tyr(3)-octreotate are useful for the diagnosis, staging and follow-up of pheochromocytomas (PHEOs) and paragangliomas (PGLs) (PPGLs). In addition to their ability to detect and localize the disease, they allow a better molecular characterization of the tumours, which is useful for planning targeted therapy with iodine-131 (131I) -labelled MIBG or with peptide receptor radionuclide therapy (PRRT) with [177Lu]-labelled DOTATATE or other related agents in patients with metastatic disease. In this review we detail the main characteristics of the radiopharmaceuticals used in the functional study of PPGLs and the role of nuclear medicine tests for initial evaluation, staging, selection of patients for targeted molecular therapy, and radiation therapy planning. It also offers a series of practical recommendations regarding the functional imaging according to the different clinical and genetic scenarios in which PPGLs occur, and on the indications and efficacy of therapy with [131I]-MIBG and 177Lu-DOTATATE.

Imaging manifestations of von Hippel-Lindau disease: an illustrated guide focusing on abdominal manifestations / Manifestações de imagem da doença de von Hippel-Lindau: um guia ilustrado das manifestações abdominais

Abstract Von Hippel-Lindau (VHL) disease is a monogenic autosomal dominant disorder with germline mutations of the VHL anti-oncogene on the short arm of chromosome 3 (3p25-26). It affects 1:36,000-50,000 individuals, with a penetrance greater than 90% at 65 years of age. Although of variable onset and presentation, with pleiotropism even among members of the same family who share a specific mutation, VHL disease usually manifests initially in young adults. It predisposes to the development of benign and malignant tumors of the central nervous system (CNS) and visceral organs. The clinical diagnosis of VHL disease can be made in the following circumstances: a) in patients with a family history of the disease and at least one of the tumors characteristic of it (e.g., retinal or CNS hemangioblastomas, clear cell renal cell carcinoma, pancreatic neuroendocrine tumors, and endolymphatic sac tumors); b) in patients with two or more CNS hemangioblastomas; c) or in patients with a retinal or CNS hemangioblastoma plus at least one visceral tumor characteristic of the disease, excluding renal and epididymal cysts. Imaging plays an important role in the diagnosis and follow-up of patients with VHL disease. This pictorial essay presents characteristic images of abdominal manifestations of VHL disease-related tumors that all radiologists should be aware of.

Resumo A doença de von Hippel-Lindau (VHL) é uma desordem autossômica dominante monogênica com mutações na linha germinativa do antioncogene VHL, no braço curto do cromossomo três (3p25-26). Afeta 1:36.000-50.000 indivíduos, com penetrância superior a 90% aos 65 anos de idade. Embora tenha início e apresentação variáveis, com pleiotropismo mesmo entre membros da mesma família que partilham uma mutação específica, usualmente manifesta-se de início em adultos jovens e predispõe ao desenvolvimento de tumores benignos e malignos no sistema nervoso central (SNC) e órgãos viscerais. Clinicamente, o diagnóstico pode ser realizado em uma das seguintes circunstâncias: a) em pacientes com história familiar de doença de VHL e pelo menos um dos tumores característicos relacionados à síndrome (como hemangioblastomas retinianos ou do SNC, carcinoma de células renais de células claras, tumores neuroendócrinos pancreáticos e tumores do saco endolinfático); b) dois ou mais hemangioblastomas do SNC; c) um hemangioblastoma retiniano ou do SNC mais pelo menos um tumor característico visceral relacionado à síndrome, excluindo-se cistos renais e epididimários. Nesse contexto, a imagem ocupa importante papel no diagnóstico e acompanhamento desses pacientes. Este ensaio iconográfico apresenta imagens características de manifestações abdominais de tumores relacionados à doença de VHL que todos os radiologistas devem conhecer.

Intraoperative adenosine for pheochromocytoma with myocardial infarction and SARS-CoV-2. Case report / Adenosina intraoperatoria para feocromocitoma con infarto miocárdico y SARS-CoV-2. Reporte de caso

Abstract Pheochromocytomas are neuroendocrine tumors capable of synthetizing, storing and releasing catecholaminergic hormones that may lead to lifethreatening hemodynamic instability. The COVID-19 pandemic has increased the risks and perioperative complexity of the patients undergoing pheochromocytoma-associated adrenalectomy. This article discusses the use of adenosine for the management of hypertensive crisis during this intervention, as well as the need to individualize the suitable timing for surgery after recent COVID-19 infection. This article discusses the case of a patient with a finding of right adrenal incidentaloma; further studies determined a metanephrines secreting pheochromocytoma. Following hospital admission for preoperative optimization, the eve of the procedure the patient developed an acute myocardial infarction and subsequently SARS-CoV-2 symptomatic infection. Intraoperatively, hypertensive peaks were managed with continuous adenosine perfusion. The patient was discharged after 48 hours. Preoperative optimization positively influences the intraoperative management of patients with pheochromocytoma. The intraoperative use of adenosine allows for adequate and safe control of hypertensive crises. Each situation must be individualized in patients pending surgery, with a recent COVID-19 infection.

Resumen Los feocromocitomas son tumores neuroendocrinos capaces de sintetizar, almacenar y liberar hormonas catecolaminérgicas que pueden provocar inestabilidad hemodinámica con compromiso vital. La pandemia por COVID-19 ha aumentado los riesgos y la complejidad perioperatoria de los pacientes sometidos a adrenalectomía por feocromocitoma. Describimos el uso de adenosina para manejar las crisis hipertensivas durante esta intervención, así como establecer la necesidad de individualizar el momento quirúrgico idóneo tras infección reciente por COVID-19. Presentamos el caso de un paciente con hallazgo de incidentaloma suprarrenal derecho cuya ampliación de estudio se orientó como feocromocitoma secretor de metanefrinas. Tras ingreso hospitalario para optimización preoperatoria, el día previo al procedimiento presentó un infarto agudo de miocardio y posteriormente una infección sintomática por SARS-CoV-2. Intraoperatoriamente se manejaron los picos hipertensivos con perfusión continua de adenosina. Tras 48 horas recibió el alta hospitalaria. La optimización preoperatoria influye positivamente en el manejo intraoperatorio de los pacientes con feocromocitoma. El uso intraoperatorio de adenosina permite un adecuado y seguro control de las crisis hipertensivas. En pacientes pendientes de cirugía con infección reciente por COVID-19 se requiere individualizar cada situación.

Síndrome coronario agudo por feocromocitoma

RESUMEN El feocromocitoma es un tumor cromafín ubicado generalmente en la glándula suprarrenal, originado en células del neuroectodermo y productor de catecolaminas. Se presenta caso clínico de varón de 56 años con antecedente de hipertensión arterial que consultó por síntomas de un síndrome coronario agudo. La cinecoronariografía descarta cardiopatía isquémica ateroesclerótica y se llegó al diagnóstico de feocromocitoma a partir de los estudios de imágenes y la anatomía patológica. El tumor fue extirpado con cirugía abierta. En la hipertensión arterial de difícil manejo y en patologías cardiacas de presentaciones atípicas, el feocromocitoma debe ser tenido en cuenta como diagnóstico diferencial.

ABSTRACT Pheochromocytoma is a chromaffin tumor generally located in the adrenal gland, originated in neuroectoderm cells and a catecholamine-producing tumor. We present a clinical case of a 56-year-old man with a history of arterial hypertension who consulted for symptoms of an acute coronary syndrome. The coronary angiography ruled out atherosclerotic ischemic heart disease and the diagnosis of pheochromocytoma was reached based on imaging studies and pathology. The tumor was removed with open surgery. In arterial hypertension that is difficult to manage and in cardiac pathologies with atypical presentations, pheochromocytoma must be taken into account as a differential diagnosis.

Adrenal pheochromocytoma: Keys to radiologic diagnosis.

Pheochromocytomas are adrenal paragangliomas. Potentially malignant, these tumors have a low incidence but clear importance. They can appear in various hereditary syndromes, especially in von Hippel-Lindau syndrome, multiple endocrine neoplasia-2 (MEN2), and familial paraganglioma syndromes. In sporadic cases, underlying genetic alterations are often found, and these findings are changing our understanding of the disease. Although these tumors can manifest with a characteristic clinical presentation, in 13.1%-57.6% of cases, it is the radiologist who first suggests the diagnosis, indicating analyses for catecholamines or nuclear medicine examinations. Radiologists should suspect a pheochromocytoma on detection of a well-delimited adrenal mass with rapid, intense enhancement that typically shows cystic and hemorrhagic phenomena, high T2 signal intensity, and the absence of macroscopic or microscopic lipids. The behavior in diffusion-weighted imaging usually does not provide very useful information. Approximately one-third of lesions show late washout similar to that seen with adenomas on CT. Percutaneous puncture should be avoided to avoid the risk of unleashing a severe hypertensive crisis.

Feocromocitoma adrenal. Claves para el diagnóstico radiológico / Adrenal pheochromocytoma: keys to radiologic diagnosis

El feocromocitoma es un paraganglioma adrenal potencialmente maligno, con baja incidencia, pero relevancia evidente. Puede aparecer en varios síndromes hereditarios, especialmente von Hippel-Lindau, neoplasia endocrina múltiple 2 y paraganglioma familiar. En casos esporádicos subyacen también frecuentemente alteraciones genéticas que están cambiando el paradigma de la enfermedad.Aunque puede tener una presentación clínica característica, en un 13,1-57,6% de los casos es el radiólogo el primero en sugerirlo, indicando determinaciones analíticas de catecolaminas o exploraciones de Medicina Nuclear.Debe sospecharse ante una masa adrenal bien delimitada con realce intenso y rápido, mostrando característicamente fenómenos quísticos y hemorrágicos, alta señal en T2 y ausencia de lípidos macro o microscópicos. El comportamiento en difusión no suele aportar información muy relevante. Aproximadamente un tercio presentan lavado tardío similar al del adenoma en TC. Debe evitarse su punción percutánea ante su sospecha, por el riesgo de desencadenar una crisis hipertensiva grave.(AU)

Pheochromocytomas are adrenal paragangliomas. Potentially malignant, these tumors have a low incidence but clear importance. They can appear in various hereditary syndromes, especially in von Hippel-Lindau syndrome, multiple endocrine neoplasia-2 (MEN2), and familial paraganglioma syndromes. In sporadic cases, underlying genetic alterations are often found, and these findings are changing our understanding of the disease.Although these tumors can manifest with a characteristic clinical presentation, in 13.1% to 57.6% of cases, it is the radiologist who first suggests the diagnosis, indicating analyses for catecholamines or nuclear medicine examinations.Radiologists should suspect a pheochromocytoma on detection of a well-delimited adrenal mass with rapid, intense enhancement that typically shows cystic and hemorrhagic phenomena, high T2 signal intensity, and the absence of macroscopic or microscopic lipids. The behavior in diffusion-weighted imaging usually does not provide very useful information. Approximately one-third of lesions show late washout similar to that seen with adenomas on CT. Percutaneous puncture should be avoided to avoid the risk of unleashing a severe hypertensive crisis.(AU)

Feocromocitoma adrenal gigante derecho. Reporte de un caso / Right giant adrenal pheochromocytoma. A case report

Introducción. El feocromocitoma es una neoplasia endocrina productora de catecolaminas, poco común, que generalmente se origina en la medula suprarrenal, y rara vez en el tejido cromafín extraadrenal, dándosele el nombre de paraganglioma. Existe una gran variedad de signos y síntomas secundarios a la secreción excesiva de catecolaminas por lo que su diagnóstico y tratamiento oportunos son fundamentales para evitar complicaciones potencialmente fatales. Caso clínico. Paciente femenina de 54 años, con dolor abdominal intermitente y con aumento progresivo, localizado en el cuadrante superior derecho. Por estudios imagenológicos se diagnosticó una gran masa suprarrenal derecha, con pruebas de laboratorio que encontraron niveles de metanefrinas y catecolaminas en orina normales. Discusión. Debido al tamaño del tumor y al íntimo contacto con las estructuras adyacentes, se realizó la resección por vía abierta, sin complicaciones y con una buena evolución postoperatoria. El informe anatomopatológico confirmó el diagnóstico de feocromocitoma suprarrenal derecho. Conclusión. Aunque poco frecuente, el feocromocitoma es una patología que se debe sospechar ante la presencia de masas suprarrenales y alteraciones relacionadas con la secreción elevada de catecolaminas. Se debe practicar el tratamiento quirúrgico de forma oportuna.

Introduction. Pheochromocytoma is a rare catecholamine-producing endocrine neoplasm that generally originates in the adrenal medulla, and rarely in extra-adrenal chromaffin tissue, giving it the name of paraganglioma. There is a wide variety of signs and symptoms secondary to excessive secretion of catecholamines, so its timely diagnosis and treatment are essential to avoid potentially fatal complications. Clinical case. A 54-year-old female patient with intermittent abdominal pain and progressive increase, located in the right upper quadrant. By imaging studies, a large right adrenal mass was diagnosed, with laboratory tests that found normal levels of metanephrines and catecholamines in urine. Discussion. Due to the size of the tumor and the intimate contact with the adjacent structures, the resection was performed by open approach, without complications and with a good postoperative evolution. The pathology report confirmed the diagnosis of right adrenal pheochromocytoma. Conclusion. Although rare, pheochromocytoma is a pathology that should be suspected in the presence of adrenal masses and changes related to elevated catecholamine secretion. Surgical treatment should be performed in a timely manner.

Anaesthetic management of a dopamine-secreting phaeochromocytoma.

Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla. Most adrenal pheochromocytomas secrete norepinephrine and epinephrine. Dopamine secreting pheochromocytomas are extremely rare and differs from classic pheochromocytomas in clinical features, posing a significant diagnostic challenge. A 41-year-old women presented to our emergency department because of acute flank colic pain and normotension. The screening abdominal computed tomography scan revealed a left adrenal mass. The laboratory test showed significantly increase in plasma dopamine and 24-h urine dopamine. During surgical removal the patient remained hypotensive requiring doses of norepinephrine. The patient presented significant hypertensive responde during direct laryngoscopy and intubation.

Hipertensión secundaria: ¿cuándo y cómo buscarla? / Secondary hypertension: when and how to look for it?

Resumen En la población hipertensa se detecta una causa subyacente en 5% a 10%. Identificar etiología y establecer un tratamiento específico reduce el riesgo de daño de órgano blanco y, en algunos casos, puede curar la enfermedad primaria y la hipertensión. Dada su baja prevalencia, no se recomienda realizar una búsqueda exhaustiva de causas secundarias en todos los pacientes hipertensos. En este artículo se analiza cuándo y cómo debe estudiarse una hipertensión secundaria.

Abstract In the hypertensive population, between 5-10%, an underlying cause is detected. Identifying the etiology and giving specific treatment reduces the risk of hypertension-mediated organ damage and, in some cases, can cure the disease and high blood pressure. Due to its low prevalence, an exhaustive search for secondary causes is not recommended in all patients with hypertension. This article will discuss when and how to study secondary hypertension.

Manejo anestésico de feocromocitoma productor de dopamina / Anaesthetic management of a dopamine-secreting phaeochromocytoma

El feocromocitoma es un tumor neuroendocrino raro que se origina en las células cromafines de la cresta neural del sistema nervioso autónomo. La mayoría de las feocromocitomas se caracterizan por secretar adrenalina y noradrenalina. Los productores de dopamina son infrecuentes y no presentan la sintomatología clínica típica, por lo que el diagnóstico puede ser complicado. Actualmente disponemos de escasa bibliografía sobre el manejo anestésico de este tipo de tumores.Presentamos el caso clínico de una mujer de 41 años que acudió a nuestro centro por dolor lumbar de tipo cólico de un mes de evolución y normotensión. Se realizó una tomografía axial computarizada abdominal que reveló masa hipercaptante en glándula suprarrenal izquierda. Los niveles de dopamina en orina y en plasma estaban elevados, los niveles de adrenalina y noradrenalina eran normales. Durante la intervención quirúrgica la paciente se mantuvo hipotensa precisando dosis de noradrenalina. Solo presentó un único pico hipertensivo durante la laringoscopia y la intubación orotraqueal.(AU)

Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla. Most adrenal pheochromocytomas secrete norepinephrine and epinephrine. Dopamine secreting pheochromocytomas are extremely rare and differs from classic pheochromocytomas in clinical features, posing a significant diagnostic challenge.A 41-year-old women presented to our emergency department because of acute flank colic pain and normotension. The screening abdominal computed tomography scan revealed a left adrenal mass. The laboratory test showed significantly increase in plasma dopamine and 24-hour urine dopamine. During surgical removal the patient remained hypotensive requiring doses of norepinephrine. The patient presented significant hypertensive responde during direct laryngoscopy and intubation.(AU)

Hipertensión maligna y feocromocitoma: reporte de un caso / Malignant hypertension and pheochromocytoma: a case report

La hipertensión arterial maligna se define por cifras extremadamente altas de presión arterial asociadas a daño orgánico. Constituye una causa de emergencia hipertensiva donde coexisten cifras elevadas de presión arterial, con hemorragia y exudados bilaterales retinianas (retinopatía hipertensiva grado III), con o sin papiledema (retinopatía hipertensiva grado IV), asociada usualmente a lesión renal o cardíaca. En un 1% de los casos es secundaria a causas endocrinológicas, entre ellas, la más frecuente: el feocromocitoma, que clásicamente se ha caracterizado por la tríada cefalea, sudoración y palpitaciones. Sin embargo, no existe un hallazgo clínico único que tenga un valor significativo en su diagnóstico. A continuación, presentamos el caso de una paciente de 23 años con emergencia hipertensiva y masa suprarrenal asociado a retinopatía hipertensiva grado IV. (AU)

Malignant arterial hypertension is defined by extremely high levels of pressure associated with organ damage. It is a cause of hypertensive emergency and is defined by the coexistence of high blood pressure and bilateral retinal haemorrhage or exudates (grade III hypertensive retinopathy), with or without papilloedema (grade IV hypertensive retinopathy) currently associated with organ damage such as renal or cardiac failure. Around 1% of malignant arterial hypertension is secondary to endocrinological causes, including the most common: pheochromocytoma, which is classically characterized by the triad: headache, sweating and palpitations. However, there is no single clinical finding that is of significant value in its diagnosis. We now present the case of a 23-year-old patient with a hypertensive emergency, an adrenal mass associated with grade IV hypertensive retinopathy. (AU)