Reporte de caso de feocromocitoma bilateral asociado a mutación del gen TMEM127. Primer caso chileno / Bilateral pheochromocytoma associates with TMEM127 gene mutation. Report of one case

Up to 40% of Pheochromocytoma/paraganglioma syndromes are associated with germline mutations. Therefore, they are considered familial and heritable. We report a 65 year old woman with hypertension, bilateral adrenal nodules found in the CT scan and elevated urinary metanephrines. Her genetic testing showed a c.117_120delGTCT TMEM127 gene mutation. She was subjected to a laparoscopic bilateral adrenal excision. After five years of follow up, no recurrence of the disease has been recorded.

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.

Strategy of reoperation for pheochromocytoma and paraganglioma / 北京大学学报(医学版)

OBJECTIVE@#To explore the surgical strategy and experience of reoperation for pheochromocytoma and paraganglioma which is very challenging.@*METHODS@#The clinical data of 7 patients with pheochromocytoma and paraganglioma who underwent reoperation in Department of Urology, Peking University Third Hospital from August 2016 to February 2021 were analyzed retrospectively. There were 4 males and 3 females, with an average age of (44.1±11.5) years (28-60 years), 6 cases on the right side and 1 case on the left side. The causes of the operations included: (1) 2 cases of tumor recurrence after resection; (2) The primary operations failed to completely remove the tumors in 3 cases, because the tumors were large and closely related to blood vessels. (3) Pheochromocytoma and paraganglioma wasn't diagnosed before primary operation, therefore, drug preparation wasn't prepared. Two cases were interrupted by severe blood pressure fluctuations during the primary operations. Imaging evaluation, catecholamine biochemical examination and adequate adrenergic α receptor blockers were administrated in all the cases. The surgical approaches included open transperitoneal surgery in 4 cases, robot-assisted laparoscopy in 1 case and retroperitoneal laparoscopy in 2 cases. The innovative techniques included mobilization of the liver, inferior vena cava transection and anastomosis, and transection of left renal vein.@*RESULTS@#The average tumor size was (8.0±3.2) cm (3.6-13.9 cm). The median interval between the reoperation and the primary operation was 9 months (IQR: 6, 19 months). The median operation time was 407 min (IQR: 114, 430 min) and the median blood loss was 1 500 mL (IQR: 20, 3 800 mL). Operations of 5 cases were performed successfully, and 1 case failed only by exploration during the operation. One case died perioperatively. There were 5 cases of intraoperative blood transfusion, the median transfusion volume of red blood cells was 800 mL (IQR: 0, 2 000 mL). One case experienced postoperative lymphorrhagia, and recovered after conservative treatment. The renal function was normal in 2 cases after resection and anastomosis of inferior vena cava or transection of left renal vein. The average postoperative hospital stay was (7.2±3.3) d (4-13 d). The median follow-up time of 6 patients was 33.5 months (IQR: 4.8, 48.0 months). The case who failed in the reoperation still survived with tumor and there was no recurrence in the rest of the patients.@*CONCLUSION@#The reoperation of pheochromocytoma and paraganglioma, which can not be resected in the primary operation or recurred postoperatively, is difficult with high risk of hemorrhage, and there is a risk of failure and perioperative death. Different surgical approaches and strategies need to be adopted based on the different situation.

Manejo perioperatorio de feocromocitoma y paraganglioma / Perioperative management of paragangliomas and pheochromocytomas

Los feocromocitomas y paragangliomas son tumores neuroendocrinos raros, caracterizados por una alta tasa de morbilidad debida a un exceso de niveles de catecolaminas. Este exceso de catecolaminas es independiente de los estresores fisiológicos. Para el diagnóstico de un feocromocitoma-paraganglioma son fundamentales las pruebas bioquímicas. Las más utilizadas son las metanefrinas fraccionadas urinarias o metanefrinas libres plasmáticas. Seguido del diagnóstico bioquímico, debe realizarse un estudio imagenológico. La evaluación del paciente con diagnóstico de feocromocitoma-paraganglioma debe realizarse teniendo presente sus principales causas de morbimortalidad perioperatoria. Las dos grandes intervenciones que han disminuido la mortalidad perioperatoria son la introducción del α bloqueo y la restauración de la volemia. El otro gran avance ha sido la introducción de la cirugía laparoscópica como el estándar de oro para el abordaje quirúrgico. En relación con el manejo intraoperatorio, no se ha identificado que alguna técnica anestésica sea superior a otra. Sí se ha logrado establecer criterios de inestabilidad hemodinámica que se correlacionan con mayor morbilidad, por lo que los principales objetivos intraoperatorios son mantener estabilidad hemodinámica. El avance en el manejo preoperatorio e intraoperatorio con la consecuente disminución en la mortalidad relacionada a esta patología ha llevado el foco al manejo postoperatorio tanto agudo como a largo plazo. También se debe considerar el riesgo de recurrencia tumoral, por lo que estos pacientes deben tener un control anual de por vida.

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, characterized by a high morbidity rate due to catecholamine excess. These high levels are independent of physiologic stressors. For the diagnosis, a biochemical workup is paramount. The most widely used are plasma-free metanephrines and urinary fractionated metanephrines. Imaging studies should be initiated once the biochemical diagnosis is established. Evaluation of the patient with pheochromocytomas and paragangliomas must be done taking into account the leading causes of perioperative morbidity and mortality. The two primary interventions that have reduced perioperative mortality are alpha-adrenergic blockade and intravascular volume normalization. Another significant advance has been the establishment of laparoscopic surgery as the gold standard for the surgical approach. No anesthetic technique has been found to be superior to another. Intraoperative hemodynamic instability has been correlated with poorer outcomes; thus one of the main intraoperative goals is maintaining hemodynamic stability. Lower morbidity and almost zero mortality rates due to preoperative and intraoperative management improvements have led to a focus on the immediate and long-term postoperative care. Anual lifelong follow-up is recommended to detect recurrent disease.

Laparoscopic management of paraganglioma in a pregnant woman: a case report

ABSTRACT Introduction: Paraganglioma is an extremely rare catecholamine-producing tumor during pregnancy. Paraganglioma carries high risks of fetal and maternal mortality during pregnancy. We report a pregnant woman with paraganglioma in the second trimester. Case Description: A 24-year-old pregnant woman presented with severe hypertension in the 17th week of gestation. Hormonal examination and Magnetic Resonance Imaging (MRI) confirmed the diagnosis of extra adrenal pheochromocytoma (paraganglioma). She underwent laparoscopic tumor excision successfully. Conclusions: A high index of suspicion is needed to diagnose paraganglioma in a pregnant patient with hypertension. Laparoscopic tumor removal for paraganglioma seems to be a feasible and safe procedure during pregnancy.

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.

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.

Caso clínico: Feocromocitoma Gigante / Case Report: Giant Pheocromocytoma

INTRODUCCIÓN: El feocromocitoma es una neoplasia neuroendocrina localizada en la médula adrenal, llamado paraganglioma cuando su ubicación es extraadrenal. Su incidencia anual es de 2-8 por millón de personas, sin predilección de sexo. Su cuadro clínico se caracteriza por: cefalea, diaforesis, taquicardia e hipertensión arterial. El tratamiento es la resección quirúrgica con una preparación preoperatoria multidisciplinaria. CASO CLÍNICO: Se trata de una paciente de 36 años, femenina con antecedentes de hipertensión arterial de un año de evolución, acompañado de cefalea, pérdida de peso, dolor abdominal, diaforesis, malestar general y otros; hospitalizada por varias ocasiones debido a crisis hipertensivas. Tensión arterial 180/120 mmHg, Frecuencia cardíaca 90/min, Índice de masa corporal 19.2 Kg/m2. Abdomen: masa palpable en hipocondrio y flaco izquierdos de aproximadamente 10 cm, no doloroso a la palpación. EVOLUCIÓN: Dentro de los exámenes complementarios, la determinación de catecolaminas en plasma fueron normales, en la tomografía computarizada se evidenció una masa retroperitoneal que involucraba riñón y suprarrenal izquierdos; ante la persistencia de hipertensión, se realiza la resección de la neoplasia por laparotomía más nefrectomía izquierda, flebotomía de cava inferior y transversectomía. Como complicación en el postoperatorio inmediato presentó hemoperitoneo requiriendo relaparotomía con esplenectomía, además adquirió neumonía la cual fue superada satisfactoriamente y fue dada el alta médica en buenas condiciones. CONCLUSIONES: El feocromocitoma es una patología infrecuente y compleja, cuya resolución quirúrgica es mandatoria, con una preparación prequirúrgica multidisciplinaria para disminuir al máximo la morbimortalidad y con un seguimiento a largo plazo en todos los casos.(AU)

INTRODUCTION: Pheochromocytoma is a neuroendocrine tumor located in the adrenal medulla or paraganglioma when its location is extraadrenal. The annual incidence is 2-8million people, no sex predilection. Clinical symptoms are characterised by: headache, diaphoresis,tachycardia, and hypertension. The treatment is surgical resection with a multidisciplinary preoperative preparation. CASE REPORT: This isa36 year old female patient,withahistory of hypertension forayear,accompanied by headache, weight loss, abdominal pain, diaphoresis, malaise and others; hospitalized for several times due to hypertensive crises. BP: 180/120 mmHg; HR: 90 per minute; BMI 19.2 kg / m2. Abdomen: palpablemass in upper quadrant and left flank of approximately 10 cm, not painful on palpation. EVOLUTION: About the examinations, the plasma catecholamines tests were normal and the CT scan showed a retroperitoneal mass which involved kidney and adrenal on left side. At the persistence of hypertension, the resection of the tumor was performed by laparotomy; also was performed left nephrectomy, phlebotomy of inferior cava and the resection ofthe transverse colon. Inthe immediatepostoperativeperiod,thepatientpresentedhemoperitoneumandasplenectomy was performed; also contracted pneumonia which was successfully treated. She left the hospital in good condition. CONCLUSIONS: The Pheochromocytoma is an infrequent and complex disease, whose surgical treatment is mandatory. A multidisciplinary preoperative preparation is important to reduce the morbidity andmortality associated and the follow-upmust be long termin all cases.(AU)

Feocromocitoma quístico gigante: reporte de un caso / Pheochromocytoma giant cystic: a case report

El feocromocitoma quístico gigante es tumor adrenal raro en el que predomina el curso asintomático; por lo que muchos de los casos no son diagnosticados hasta el momento de la cirugía. La simple movilización del tumor se asocia con el paso a la sangre de grandes cantidades de catecolaminas y a una elevada morbimortalidad.; por esta razón la cirugía per se y su manejo perioperatorio constituyen un enorme desafío. En este artículo se presenta el caso de un feocromocitoma gigante maligno (35 cm) que ocupaba todo el hemiabdomen derecho. Aun con el diagnóstico preoperatorio de feocromocitoma, el bloqueo farmacológico preoperatorio y las medidas intraoperatorias, el paciente falleció poco antes de que finalizara la cirugía.

The giant cystic pheochromocytoma is a rare adrenal tumor in the predominantly asymptomatic course; so many cases are not diagnosed until the time of surgery. The simple mobilization of the tumor is associated with the passage to the blood of large amounts of catecholamines and high morbidity and mortality. So the surgery itself and perioperative management are a huge challenge. This article describes the case of a malignant giant pheochromocytoma (35 cm) which occupied the entire right abdomen. Even with the preoperative diagnosis of pheochromocytoma, pharmacological blockade preoperative and intraoperative measures, the patient died shortly before the end of surgery.

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.

Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders

OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

Presentation and surgery outcomes in elderly with pheocromocytoma: a comparative analysis with young patients

ABSTRACT Purpose: To evaluate the presentation and early surgical outcomes of elderly patients undergoing adrenalectomy for phaeochromocytoma. Patients and Methods: A retrospective search was performed of our adrenal disorders database for patients who underwent surgery for phaeochromocytoma or paraganglioma between 2009 and 2014. Patients >60 years old were classified as elderly. The clinical manifestations, intraoperative course, and early postoperative outcomes of elderly patients were compared to those of younger individuals (<60 years old). Results: The mean (±standard deviation) age in the older (n=10) and younger (n=36) groups was 69.6±5.3 years and 34.0±12.9 years. Germ-line mutations were more common in younger patients (50.0% versus 0%; p=0.004), whereas incidental lesions were more common in the elderly (40.0% versus 5.3%; p=0.003). In both groups, surgery was most commonly performed by videolaparoscopy (90% in the elderly and 82% in the younger group), with similar intraoperative anesthetic and surgical outcomes. Postoperatively, the older group more commonly received vasoactive drugs (60.0% versus 10.5%; p<0.001) and had a longer intensive care unit stay (3.1±2.8 versus 1.4±1.0 days; p=0.014), more clinical complications (60% versus 18.9%; p=0.01), and longer hospital stay (10.2±8.4 versus 5.7±4.9 days; p=0.028). Conclusions: Although all patients received the same preoperative preparation, the elderly group exhibited a slower and more complicated recovery after adrenalectomy. Meticulous perioperative care should be used in the elderly when treating phaeochromocytoma; nevertheless, adrenalectomy is a relatively safe procedure in this patient population.

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)

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 maligno: comunicación de 2 casos tratados con cirugía de mínima invasión / Malignant pheochromocytoma treated with minimally invasive surgery

Introducción: El Feocromocitoma es un tumor de células cromafines productor de catecolaminas. Su variedad maligna es muy poco frecuente. Objetivo: Describir 2 casos de Feocromocitoma maligno tratados con cirugía mínimamente invasiva. Casos clínicos: Presentamos 2 pacientes de sexo femenino, de 31 y 51 años respectivamente. La primera con historia de hipertensión arterial de larga data y uso de múltiples fármacos. La segunda paciente debuta con dolor abdominal. En ambos casos los estudios radiológicos y metabólicos confirmaron el diagnóstico de Feocromocitoma. A la primera paciente se le realizó un procedimiento laparoscópico puro, mientras que a la segunda paciente se le realizó un procedimiento laparoscópico asistido por robot. En ambos casos fue necesario realizar adrenalectomía en bloque con el riñón, por compromiso del hilio renal. No hubo complicaciones severas intra ni post-operatorias, como tampoco conversión a cirugía abierta. La histopatología confirmó el diagnóstico de Feocromocitoma maligno con metástasis ganglionares regionales y márgenes quirúrgicos negativos. Ambas se mantienen en control, sin evidencia de recurrencia. Conclusión: El Feocromocitoma maligno puede ser manejado de forma mínimamente invasiva, con morbilidad y resultados oncológicos comparables a cirugía abierta.

Introduction: Pheochromocytoma is a tumor derived from chromaffin cells that produces cathecolamines. Malignant pheochromocytomas are extremely rare. Aim: To describe 2 cases of malignant Pheochromocytoma treated with pure laparoscopic and robot assisted surgery. Clinical cases: Two female patients, 31 and 51 years of age, presented with a history of high blood pressure and abdominal pain. Radiologic and metabolic studies confirmed the diagnosis of Pheochromocytoma in both patients. A pure laparoscopic approach was performed in the first patient while a robotic assisted laparoscopy was done in the second patient. In both cases, adrenalectomy with in bloc nephrectomy was performed due to compromise of the renal sinus. There were no complications or conversion to open surgery. Histopathology confirmed the diagnosis of malignant Pheochromocytoma with metastasis to regional nodes and negative surgical margins. At follow-up, there is no evidence of recurrence. Conclusion: Malignant Pheochromocytoma can be treated with minimally invasive surgery, with morbidity and oncological outcomes similar to open approach.

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.

Pheochromocytoma mimicking both acute coronary syndrome and sepsis: a case report

To present an atypical manifestation in a patient with pheochromocytoma. A 48-year-old man presented with chest pain, fever and leukocytosis. Elevated cardiac biomarkers and diffuse ST-T abnormalities on electrocardiography suggested myocardial infarction. However, coronary angiography showed normal coronary arteries. Abdominal computed tomography revealed a left adrenal tumor of 6.7 x 6.8 cm. Paroxysmal fluctuation of blood pressure raised the suspicion of pheochromocytoma, which was further supported by elevated urine catecholamine levels. He underwent left adrenalectomy and pathological findings confirmed the diagnosis. Pheochromocytoma should be considered as part of the differential diagnosis in a patient with symptoms suggestive of both acute coronary syndrome and sepsis

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)

Retroperitoneoscopic adrenalectomy in pheochromocytoma

Since the first laparoscopic adrenalectomy, the technique has evolved and it has become the standard of care for many adrenal diseases, including pheochromocytoma. Two laparoscopic accesses to the adrenal have been developed: transperitoneal and retroperitoneal. Retroperitoneoscopic adrenalectomy may be recommended for the treatment of pheochromocytoma with the same peri-operative outcomes of the transperitoneal approach because it allows direct access to the adrenal glands without increasing the operative risks. Although technically more demanding than the transperitoneal approach, retroperitoneoscopy can shorten the mean operative time, which is critical for cases with pheochromocytoma where minimizing the potential for intra-operative hemodynamic changes is essential. Blood loss and the convalescence time can be also shortened by this approach. There is no absolute indication for either the transperitoneal or retroperitoneal approach; however, the latter procedure may be the best option for patients who have undergone previous abdominal surgery and obese patients. Also, retroperitoneoscopic adrenalectomy is a good alternative for treating cases with inherited pheochromocytomas, such as multiple endocrine neoplasia type 2A, in which the pheochromocytoma is highly prevalent and frequently occurs bilaterally.