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.

Ruptured abdominal aortic aneurysm with a suprarenal tumor

Abstract This paper presents a case study of a patient that underwent surgery for a ruptured abdominal aneurysm. The postoperative course was complicated by resistant hypertension and tachycardia. A suprarenal mass was detected in the computed tomography scan with radiological suspicion of pheochromocytoma. Few cases of pheochromocytoma coexisting with aneurysms have been reported. Management of cardiovascular stability is crucial in such cases. Despite the lack of evidence, pheochromocytomas might have a role in the etiology of aortic aneurysms.

Síndrome del dedo azul como manifestación de feocromocitoma / Blue toe syndrome as a clinical finding of pheochromocytoma

Los feocromocitomas son tumores que proceden de las células cromafines del sistema nervioso simpático y actúan sintetizando y liberando catecolaminas. Suelen presentarse entre la cuarta y quinta década de la vida y tienen presentaciones clínicas muy diversas. Ocurren solamente en 0.1-0.2% de la población hipertensa, constituyen una causa tratable y curable de hipertensión arterial, así como de otras manifestaciones derivadas de la liberación incontrolada de catecolaminas. La isquemia arterial periférica secundaria a la liberación masiva de aminas por un feocromocitoma es muy infrecuente. Aquí se presenta un caso clínico de feocromocitoma manifestado como síndrome del dedo azul en un paciente con pulsos distales conservados y el antecedente de mal control tensional a pesar de tratamiento con dos fármacos.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Laparoscopic approach to pheochromocytoma in pregnancy: case report

ABSTRACT A 32-year-old 22-week pregnant hypertensive woman with sporadic episodes of headaches, sweating, and facial flushing was diagnosed with pheochromocytoma through biochemical and imaging tests. Perioperative management included a multidisciplinary approach, symptom stabilization with α blockade followed by β blockade, and tumor resection by laparoscopic adrenalectomy at 24 weeks gestation. The diagnosis was confirmed by histopathological examination and immuno-histochemistry tests. The decision for surgical removal of the tumor was based on maternal symptoms, tumor size, gestational age, the possibility of doing a laparos-copy, and the expertise of the surgical team.

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.

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. .

Hipertensión arterial secundaria en el adulto: evaluación diagnóstica y manejo / Secondary arterial hypertension in adults: diagnostic assesment and management

La Hipertensión arterial (HTA) es un grave problema de salud pública mundial. En efecto, sus complicaciones causan anualmente 9,4 millones de muertes. La HTA también es un problema de salud de alto impacto en Chile. De hecho, la Encuesta Nacional de Salud (ENS) 2009-2010 del Ministerio de Salud, reportó una prevalencia del 26,9%. La HTA se define como una Presión arterial sistólica (PAS) 140mmHg y/o una Presión arterial diastólica (PAD) 90mmHg. Tradicionalmente, se ha clasificado la HTA en primaria o esencial, que agrupa a más del 90% de los hipertensos adultos; y en secundaria, que reúne a menos del 10% de los hipertensos. En la evaluación inicial de un paciente con HTA, se debe: Confirmar el diagnóstico; 2) Detectar causas de HTA secundaria, y 3) Evaluar riesgo cardiovascular (CV), daño orgánico y comorbilidades. Para ello, se necesita determinar la Presión Arterial (PA) y la historia clínica, que incluya antecedentes familiares, examen físico, pruebas de laboratorio y pruebas diagnósticas adicionales. En un pequeño porcentaje de adultos con HTA, se puede identificar una causa específica y potencialmente reversible; no obstante, debido a su elevada prevalencia, las formas secundarias pueden afectar a millones de pacientes en todo el mundo. Se puede sospechar una forma secundaria de HTA por un alza marcada de la PA, la aparición o empeoramiento repentinos de una HTA, una mala respuesta de la PA al tratamiento farmacológico y por un daño orgánico desproporcionado para la duración de la HTA. Si la evaluación inicial hace pensar que el paciente tiene una HTA secundaria, entonces se debe tener en consideración las causas más relevantes, que se describen en este artículo.

Arterial hypertension is a serious public health problem worldwide. Indeed, its complications cause 9.4 million deaths annually. Hypertension is also a health problem with high impact in Chile. In fact, the National Health Survey 2009-2010, conducted by the Ministry of Health, showed a prevalence of 26.9%. Arterial hypertension is defined as systolic blood pressure (SBP) 140mmHg and/or diastolic blood pressure (DBP) 90mmHg. Traditionally, hypertension has been classified into primary or essential, which represents over 90% of adults with hypertension; and secondary, which includes less than 10% of hypertensive patients. The initial evaluation of a patient with hypertension should: 1) Confirm the diagnosis of hypertension; 2) Detect causes of secondary hypertension; and 3) Assess cardiovascular risk, organ damage (OD) and concomitant clinical conditions. This calls for blood pressure (BP) measurement, medical history including family history, physical examination, laboratory investigations and further diagnostic tests. A specific, potentially reversible cause of BP elevation can be identified in a relatively small proportion of adult patients with hypertension. However, because of the overall high prevalence of hypertension, secondary forms can affect millions of patients worldwide. A secondary form of hypertension can be indicated by a severe elevation in BP, sudden onset or worsening of hypertension, poor BP response to drug therapy and OD disproportionate to the duration of hypertension. If the initial assessment suggests that the patient has a secondary hypertension, then you should take into consideration the relevant causes, which are described in this article.

A functioning adrenal adenoma and pheochromocytoma in the same adrenal gland: two discrete adrenal incidentalomas

No abstract available.

Hipertensión de causa adrenal y embarazo / Hypertension of adrenal origin and pregnancy

La hipertensión arterial de causa adrenal asociada al embarazo es infrecuente. Su presencia genera una elevada morbilidad y mortalidad materna y fetal. Los cambios de la fisiología endocrina que acontecen en esta etapa, modifican el cuadro clínico y bioquímico de las enfermedades adrenales causantes de hipertensión. El objetivo de esta revisión es brindar información actualizada sobre el hipercortisolismo endógeno, el aldosteronismo primario y el feocromocitoma en la gestación -a los profesionales de la salud vinculados con la atención a embarazadas- que les permita diagnosticar y tratarlas temprana y adecuadamente mediante la aplicación del método clínico. Se revisa la literatura y se exponen los avances médicos en el tema(AU)

Blood hypertension of adrenal origin associated to pregnancy is unfrequent and generates high maternal and fetal morbidity and mortality. Changes in the endocrine physiology during this phase modify the clinical and biochemical picture of hypertension-causing adrenal diseases. The objective of this review was to provide health professionals in charge of pregnancy care with updated information on endogenous hypercortisolism, primary aldosteronism and pheochromocytoma in pregnancy. This will allow them to diagnose and treat this disorder early and adequately by means of the clinical method. Relevant literature was reviewed and the latest medical advances in this field were presented(AU)

Oclusión intestinal en una paciente portadora de un Feocromocitoma no diagnosticado. Manejo anestésico / Intestinal occlusion in a patient with an undiagnosed pheochromocytoma. Anesthetic management

El feocromocitoma es un tumor productor de catecolaminas que procede de las células cromafines del sistema nervioso simpático que puede causar hipertensión severa entre otros trastornos sistémicos. Pueden ser esporádicos o encontrarse asociados a varias enfermedades genéticas: neoplasia endocrina múltiple tipo 2, enfermedad de von Hippel-Lindau, neurofibromatosis de tipo 1 y paraganglioma familiar. Cuando un paciente en el que se sospecha la presencia de un feocromocitoma se presenta con una ugencia quirúrgica representa un gran desafío para el anestesiólogo, ya que en esta situación la mortalidad aumenta notablemente. Presentamos el caso de una paciente que resultó ser portadora de un feocromocitoma, integrando un síndrome MEN 2b con masas suprarrenales bilaterales que se presentó en la urgencia con una oclusión intestinal con una crisis hipertensiva severa con edema agudo de pulmón.

Pheochromocytoma is a tumor catecholamine-producing derived from chromaffin cells of the sympathetic nervous system that can cause severe hypertension among other systemic disorders. They may be sporadic or be associated with several genetic diseases: multiple endocrine neoplasia type 2, von Hippel-Lindau, neurofibromatosis type 1 and familial paraganglioma disease. When a patient who is suspected the presence of a pheochromocytoma presents with a surgical urgency represents a great challenge for the anesthesiologist, since in this situation the mortality increases significantly. We report the case of a patient who was found to be carrying a pheochromocytoma, integrating a MEN 2b syndrome with bilateral adrenal masses showed the urgency with bowel obstruction with severe hypertensive crisis with acute pulmonary edema.

Feocromocitoma é um tumor produtor de catecolamina derivada de células cromafins do sistema nervoso simpático que pode causar hipertensão grave entre outros distúrbios sistémicos. Eles podem ser esporádica ou estar associada a várias doenças genéticas: neoplasia endócrina múltipla tipo 2, von Hippel-Lindau, a neurofibromatose tipo 1 e doença paraganglioma familiar. Quando um paciente no qual a presença de um feocromocitoma suspeito apresenta-se com um ugencia cirúrgica representa um grande desafio para o anestesiologista, uma vez que nesta situação os mortalidade aumenta significativamente. Relatamos o caso de um paciente que foi encontrado carregando um feocromocitoma, integrando MEN 2b síndrome com massas adrenais bilaterais mostrou a urgência com obstrução intestinal com crise hipertensiva grave com edema pulmonar agudo.

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.

Miocardiopatía dilatada y feocromocitoma / Dilated myocardiopathy and pheochromocytoma

El feocromocitoma, un tumor de baja incidencia con un comportamiento benigno en la mayoría de los casos, puede ser el responsable de extensas manifestaciones cardiovasculares. Las manifestaciones sobre el miocardio pueden ser de profundas consecuencias, pues llegan a producir una miocardiopatía dilatada con insuficiencia cardiaca severa. La exéresis del tumor suele ser curativa en el 90 por ciento de los casos y el manejo perioperatorio muy complejo. Se presenta un paciente joven portador de un feocromocitoma, que sometido a la larga acción de las catecolaminas llegó a desarrollar una miocardiopatía dilatada con criterio de trasplante cardiaco. La cirugía adrenal fue curativa en nuestro paciente(AU)

Pheochromocytoma, a low incidence tumor of benign behavior in most of cases, can cause extensive cardiovascular manifestations. These manifestations on the myocardium can have deep consequences since they may lead to dilated myocardiopathy with severe heart failure. The excision of tumor may be the curative solution for 90 percent of cases but the perioperative management is very complex. This is the case of a young patient who carries pheochromocytoma and after a long-acting catecholemine-based treatment, he developed dilated myocardiopathy, and the medical criterion was that he required heart transplantation. The adrenal surgery was successfully healing in our patient(AU)

Severe Posterior Reversible Encephalopathy in Pheochromocytoma: Importance of Susceptibility-Weighted MRI

Pheochromocytoma is a rare cause of hypertension in children. Hypertension is one of the common reasons of posterior reversible encephalopathy. Intracerebral hemorrhage is a serious and unexpected complication of hypertensive encephalopathy due to pheochromocytoma, and very rarely seen in the childhood. Intracerebral hemorrhages should be searched if there are hypertensive reversible signal changes on the brain. Susceptibility weighted imaging (SWI) is a more sensitive method than conventional MRI when demonstrating cerebral microhemorrhagic foci. This is the first report of SWI findings on intracerebral hemorrhages in basal ganglia, brain stem and periventricular white matter due to hypertensive encephalopathy in a child with pheochromocytoma.

Hypertensive crisis in a patient with thyroid cancer.

Phaeochromocytomas may be discovered incidentally when patients present with hypertensive crisis during general anaesthesia. A 49-year-old man underwent thyroidectomy 25 years ago and was diagnosed to have spindle cell carcinoma of the thyroid. He presented with recent onset of hoarseness of voice and was found to have a vocal cord nodule. He developed a hypertensive crisis during surgery. He was subsequently evaluated and found to have bilateral phaeochromocytoma. Further evaluation revealed a RET proto-oncogene mutation at codon 634 consistent with multiple endocrine neoplasia (MEN)-2A.

Anestesia para suprarrenalectomia em pacientes com feocromocitoma bilateral: relato de dois casos e considerações sobre o tema / Anesthesia for adrenalectomy in patients with bilateral pheochromocytoma: a two-case report and remarks on the topic

O feocromocitoma é um tumor raro, sendo sua incidência estimada em um a dois casos por 100.000 adultos. Ocorrem em aproximadamente 0,1% da população hipertensa, sendo importante causa de hipertensão arterial grave corrigível. O tratamento cirúrgico, com retirada total de todos os focos de tecido tumoral, constitui-se no único tratamento definitivo do feocromocitoma e pode ser realizado por via aberta ou videolaparoscópica. O preparo pré-operatório é realizado com o objetivo de tratar a hipertensão arterial, evitar a ocorrência de paroxismos e de corrigir uma eventual hipovolemia. Se isso não for feito, os pacientes correm o risco de desenvolver hipotensão importante e mesmo choque hipovolêmico após a retirada do tumor e consequente desaparecimento da vasoconstrição. Durante o procedimento cirúrgico, seja ele aberto ou laparoscópico, a pressão arterial média, a pressão venosa central, o ritmo e a frequência cardíaca devem ser continuamente monitorizados. As reações hipertensivas que acontecem, inevitavelmente, durante o manuseio cirúrgico do tumor devem ser tratadas com a infusão endovenosa de drogas de ação imediata, como o alfa-bloqueador adrenérgico fentolamina ou o vasodilatador de ação direta, nitroprussiato de sódio. Taquicardia e arritmias devem ser tratadas com a administração endovenosa de beta-bloqueadores. A administração de volume, principalmente após a retirada do tumor, deve ser efetuada quando os níveis de pressão arterial média e de pressão venosa central, associados a parâmetros clínicos e laboratoriais, indicarem a existência de hipovolemia. A maioria dos pacientes com hipotensão no pós-operatório responde bem à administração de volume, sendo raramente necessárias drogas vasoativas. Outra ocorrência possível, mas menos provável, nas primeiras 24 a 48 horas do pós-operatório é a hipoglicemia, sendo recomendado controle de glicemia capilar nas primeiras 48 horas do pós-operatório. O objetivo deste artigo é apresentar dois casos de ressecção de feocromocitoma realizados em nosso serviço e com diferentes evoluções e discutir os cuidados perioperatórios para esse tipo de cirurgia.(AU)

Pheochromocytoma is a rare tumor with estimated incidence of 2 cases per 100,000 adults. It affects approximately 0.1% of the hypertensive population, and is an important cause of a correctable form of severe arterial hypertension. Surgery to remove every focus of tumoral tissue is the only definitive treatment and can be performed either by incision or videolaparoscopy. Preoperative care is provided to treat arterial hypertension, avoid paroxysms, and correct eventual hypovolemia. Otherwise, patients may develop considerable hypotension and even hypovolemic shock after tumor removal and eventually loss of vasoconstriction. Irrespective of the type of surgery, mean arterial pressure, central venous pressure, and heart rhythm and rate must be continuously monitored during procedures. The hypertensive reactions that inevitably take place during surgical handling of the tumor must be treated with intravenous infusion of rapid acting drugs, such as the adrenergic alpha-blocker phentolamine or the direct action vasodilator sodium nitroprusside. Tachycardia and arrhythmia must be treated with intravenous infusion of beta-blockers. Administration of volume, especially after tumor removal, must be carried out when the levels of mean arterial pressure and central venous pressure, associated with clinical and laboratorial parameters, point to hypovolemia. Most patients with postoperative hypotension react well to the administration of volume, and vasoactive drugs are rarely needed. It is also possible, although usually unlikely, that patients have hypoglycemia in the first 24-48 hours after surgery; capillary blood glucose testing is thus recommended within the first 48 hours after surgery. This paper aims to report on two cases of pheochromocytoma resection carried out at our health care center, which progressed differently, and to discuss perioperative care issues for this type of surgery.(AU)

Feocromocitoma: presentación de un caso / Pheochromocytoma: case report

Se presenta el caso de una paciente femenina de 35 años de edad, portadora de un tumor adrenal izquierdo diagnosticado durante un estudio ecográfico. Las características morfológicas y los parámetros clínicos y de laboratorio sugirieron la existencia de un feocromocitoma. La paciente fue intervenida quirúrgicamente y la cirugía confirmó dicho diagnóstico.