Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications

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

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

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

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.

Chronic kidney disease after adrenalectomy in a patient with primary aldosteronism / 北京大学学报(医学版)

We report one case of estimated glomerular filtration rate (eGFR) decline after taking unilateral adrenalectomy due to aldosterone adenoma. A 60-year-old male with 23-year history of hypertension was reported to the endocrinologist due to hypokalemia (serum potassium 3.01 mmol/L). Urine microalbumin/creatinine (ALB/CR) was 70.15 mg/g, serum creatinine was 82 μmol/L and eGFR was 89.79 mL/(min·1.73 m2). Random serum aldosterone was 172.2-203.5 ng/L, and random plasma rennin activity was 0-0.17 μg/(L·h). His captopril challenge test suggested that his aldosterone le-vels were suppressed by 8% (< 30%) and the adrenal enhanced computed tomography scan revealed a left adrenal tumor. The patient was diagnosed with primary hyperaldosteronism (PA), aldosterone adenoma and underwent left laparoscopic adrenalectomy. Histological examination confirmed adrenal cortical adenoma. One week after the operation, his serum creatinine was increased to 127 μmol/L compared with preoperative level; eGFR was 32.34 mL/(min·1.73 m2). His systolic blood pressure (SBP) was 110 mmHg and diastolic blood pressure (DBP) was 60 mmHg (hypotensive drugs discontinued), and serum potassium level was 5.22 mmol/L. At the end of the 2-year follow up, the serum creatinine of this patient remained at 109-158 μmol/L and eGFR fluctuated from 63.28-40.12 mL/(min·1.73 m2). PA is one of the most common causes of secondary hypertension. Several studies have reported renal function deterioration of PA patients after unilateral adrenalectomy, like the patient in this article. Age, preoperative plasma aldosterone concentration, albuminuria and preoperative potassium level might be significant predictors of a decrease in the eGFR. Growing evidence suggests that aldosterone could contribute to structural kidney damage, arterial injury and hemodynamic disorder. At the same time, patients with PA exhibit glomerular hyperfiltration and glomerular vascular hypertension, leading to the misinterpretation of renal function in PA patients as subtle kidney damage may be masked by the glomerular hyperfiltration before treatment. After a unilateral adrenalectomy, glomerular hyperfiltration by aldosterone excess is resolved and renal damage can be unmasked. In conclusion, kidney function deterioration after adrenalectomy can be detected in some patients with PA. Thus, accurate evaluation of kidney function in patients with PA may be essential, especially for those with preoperative risk factors for postoperative renal impairment. After unilateral adrenalectomy, close monitoring of renal function and adequate management are required for PA patients.

Adrenal cavernous hemangioma: A case report and literature review / 北京大学学报(医学版)

A 57-year-old male patient was referred to our department with complaints of his right adrenal gland occupancy and hypertension about 6 months. When admitted to the hospital, the blood pressure was about 160/100 mmHg, and the heart rate was 110 beats/min. He was no obvious obesity, acne, abnormal mood, without weakness of limbs, acral numbness, palpitation and headache. He presented with type 2 diabetes for more than 3 years, with oral administration of metformin enteric coated tablets and subcutaneous injection of insulin glargine to control blood glucose, and satisfied with blood glucose control. Enhanced CT showed that: the right adrenal gland showed a kind of oval isodense, slightly hypodense shadow, the edge was clear, lobular change, the size was about 5.8 cm×5.4 cm, uneven density, there were nodular and strip calcification, round lipid containing area and strip low density area, and the CT value of solid part was about 34 HU. Enhanced scan showed heterogeneous nodular enhancement in the solid part of the right adrenal gland, nodular enhancement could be seen inside. The CT values of solid part in arterial phase, venous phase and delayed phase were 45 HU, 50 HU and 81 HU, respectively. Considering from the right adrenal gland, cortical cancer was more likely. No obvious abnormality was found in his endocrine examination. After adequate preoperative preparation, retroperitoneal laparoscopic adrenalectomy was performed under general anesthesia. During the operation, the 6 cm adrenal tumor was closely related to the inferior vena cava and liver, and after careful separation, the tumor was completely removed and normal adrenal tissue was preserved. The operation lasted 180 min and the blood loss was 100 mL, and the blood pressure was stable during and after the operation. There was no obvious complication. The results of pathological examinations were as follows: the size of the tumor was 7.5 cm×6.0 cm×3.5 cm, soft, with intact capsule and grayish-red cystic in section. Pathological diagnosis: (right adrenal gland) cavernous hemangioma, secondary intravascular thrombosis, old hemorrhagic infarction with calcification and ossification. After 6 months of observation, no obvious complications and tumor recurrence were found. In summary, cavernous hemangioma of adrenal gland is a rare histopathological change. Its essence is a malformed vascular mass. Blood retention is the cause of thrombosis and calcification in malformed vessels. The imaging findings were inhomogeneous enhancement of soft tissue masses, and the adrenal function examination showed no obvious abnormalities. Retroperitoneal laparoscopic surgery is feasible after adequate preoperative preparation. It is difficult to diagnose the disease preoperatively and needs to be confirmed by postoperative pathology.

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.

Challenging risk factors for right and left laparoscopic adrenalectomy: A single centre experience with 272 cases

ABSTRACT Purpose This study aimed to compare perioperative and postoperative results of right and left laparoscopic adrenalectomy (LA), and to evaluate the impact of challenging factors on these outcomes. Materials and Methods A total of 272 patient's medical records that underwent single side LA between October 2006 and September 2017 were retrospectively reviewed. The patients were divided into 2 groups according to operation side. Moreover, pheochromocytoma, metastatic masses and adrenal lesions >5cm in size were considered to be difficult adrenalectomy cases and the outcomes of these cases were compared between two groups. Results 135 patients (49.6%) underwent right LA and 137 patients (50.4%) underwent left LA. Operation time, estimated blood loss (EBL) and hospitalization time were similar between the groups (p=0.415, p=0.242, p=0.741, respectively). Although EBL was higher on the right side than the left (p=0.038) in the first 20 cases, after this learning period has been completed, there was no significant difference between the groups. In patients with pheochromocytoma, metastatic mass and a mass >5cm in size, despite bleeding complications were clinically higher on the right side, this difference was not statistically significant. Conclusions During the learning period of LA, EBL is higher on the right side. Due to the greater risk of bleeding complications on the right side even on the hands of experienced surgeons, extra care and preoperative planning are required in patients with pheochromocytoma, metastatic masses and masses >5cm in size.

Diagnóstico diferencial y cirugía mínimamente invasiva de un tumor suprarrenal de hallazgo antenatal / Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.

Abstract: Introduction: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. Objective: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. Case report: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. Conclusions: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.

Total and partial laparoscopic adrenalectomy

The Guidelines Project, an initiative of the Brazilian Medical Association, aims to combine information from the medical field in order to standardize producers to assist the reasoning and decision-making of doctors. The information provided through this project must be assessed and criticized by the physician responsible for the conduct that will be adopted, depending on the conditions and the clinical status of each patient.

Robotic excision of complex adrenal mass with retrocaval extension and encasement of renal hilum with renal preservation

ABSTRACT Objective: The purpose of this video is to present robotic excision of a complex adrenal mass with retrocaval extension and encasement of renal hilum in a 16 year old boy. Biochemical screening was negative for metabolically active component. Computerized tomographic scan with contrast revealed a homogenous mass of approximately 10.8 cm x 6.2 cm x 4.2 cm in the suprarenal area on right side that was extend-ing behind inferior vena cava and encasing renal hilar vessels. Imaging findings were that of a classical ganglioneuroma. Material and methods: Robot assisted laparoscopic adrenalectomy with sparing of renal hilar vasculature was performed. With patient in lateral position, five ports were used, including one for liver retraction. Da Vinci® system with four arms was docked from over the right shoulder. The displaced renal hilar structures were identified by opening Gerota's fascia. Mass was dissected completely and removed through Pfan-nensteil incision. Results: Duration of procedure was 345 minutes and console time was 290 minutes. Blood loss was 250 mL. Post-operative renal doppler showed normal blood flow. He was discharged on post-operative day three. Histopathologic examination of specimen revealed ganglioneuroma arising from adrenal gland. Conclusion: Ganglioneuroma is a rare adrenal tumor with good prognosis on surgical removal. The advent of robotic surgery has made complex surgical procedures involving vital structures like inferior vena cava be performed using minimally invasive techniques without compromising oncologic principles.

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.

Cirugía del incidentaloma suprarrenal / Adrenal incidentaloma surgery

Introducción: la incidencia de incidentalomas suprarrenales ha aumentado con el uso de técnicas modernas de imagen y la indicación quirúrgica se basa en la sospecha de malignidad o hiperfunción. Objetivos: revisar una casuística de incidentalomas suprarrenales operados. Pacientes y método: 16 pacientes operados entre los años 2010 y 2017 en dos instituciones de asistencia médica. Se describen aspectos diagnósticos, técnica operatoria y morbimortalidad de los procedimientos. esultados: la mayoría de los procedimientos se realizaron por abordaje laparoscópico, destacándose tres complicaciones intraoperatorias, sin mortalidad en la serie. La lesión más frecuentemente encontrada fue el adenoma no funcionante. Discusión: la llave diagnóstica, clave en la decisión de seguir u operar un incidentaloma suprarrenal, es la imagenología. El protocolo de seguimiento continúa siendo objeto de debate actualmente, así como la indicación de cirugía en lesiones funcionantes subclínicas. Conclusiones: la suprarrenalectomía laparoscópica es una cirugía segura, con cifras de morbimortalidad en el rango descrito por la literatura.

Background: the incidence of adrenal incidentalomas has increased with the use of modern imaging techniques and surgical indication is based on suspected malignancy or hyperfunction. Objective: to review a casuistry of surgically treated adrenal incidentalomas. Method: 16 patients operated between 2010 and 2016 in two health care institutions. The study describes diagnostic aspects, operative techniques and morbimortality of the procedures. Results: most of the procedures were performed by laparoscopic approach, 3 intraoperative complications arose, there being no deaths in the series. The most frequently encountered lesion was non-functioning adenoma. Discussion: imaging constitutes the diagnostic key which results essential to make a decision as to whether to follow or operate an adrenal incidentaloma. Follow-up protocols are still under discussion, as is indication of surgery in subclinical functional lesions Conclusions: laparoscopic adrenalectomy is a safe surgery, with morbidity and mortality rates which lie within the range described by relevant literature.

Introdução: a incidência de incidentalomas suprarrenais aumentou com o uso de técnicas modernas de imagem e a indicação de cirurgia está baseada na suspeita de malignidade ou hiperfunção. Objetivos: revisar una casuística de incidentalomas suprarrenais operados. Pacientes e método: 16 pacientes operados no período 2010-2017 emduas instituições de assistência médica. Foram descritos os aspectos diagnósticos, técnica operatória e morbimortalidade dos procedimentos. Resultados: a maioria dos procedimentos foram realizados por laparoscopia, registrando-se 3 complicações intraoperatórias, sem mortalidade na série. A lesão mais frequentemente encontrada foi o adenoma não funcionante. Discussão: os aspectos diagnósticos de um incidentaloma suprarrenal, fundamentais na tomada de decisão entre fazer um seguimento ou uma cirurgia, são as imagens. O protocolo de seguimento continua sendo causa de debate atualmente, como também a indicação de cirurgia nas lesões funcionantes subclínicas. Conclusões: a suprarrenalectomia laparoscópica é uma cirurgia segura, com valores de morbimortalidade dentro dos intervalos descritos na literatura.

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.

Adrenalectomy in dogs: retrospective study of 13 cases performed in Universidade Anhembi Morumbi Veterinary Hospital (2012-2015) / Adrenalectomia em cães: estudo retrospectivo de 13 casos realizados no hospital veterinário da Universidade Anhembi Morumbi (2012 a 2015)

Adrenalectomy is the most appropriate treatment for unilateral adrenal tumors. This study aimed at describing the epidemiological characteristics and perioperative behavior of canine patients submitted to adrenalectomy at Anhembi Morumbi Veterinary Hospital. Out of 13 dogs, eight were pure breeds and five were mixed breeds; 12 females, aged 9.5 ± 2.5 years old. Regarding the tumors, seven were located on the right and histopathological analysis revealed cortical adenoma in 11 and adenocarcinoma in only two dogs. Two cases had hypercortisolism recurrence associated with hyperplasia in the contralateral adrenal, as confirmed by ACTH stimulation test. The results of this study indicate that adrenalectomy is a safe procedure with few perioperative complications, despite the possibility of hypercortisolism recurrence.(AU)

Adrenalectomia é o tratamento mais indicado para neoplasias adrenais unilaterais. Objetivou-se descrever os aspectos epidemiológicos e o comportamento perioperatório em cães submetidos a adrenalectomia no Hospital Veterinário Anhembi Morumbi. Dos 13 casos, oito eram cães de raças puras e cinco eram SRD; 12 eram fêmeas e de idade de 9,5 ± 2,5 anos. Das massas adrenais, sete eram em lado direito. Os exames histopatológicos revelaram adenoma cortical em 11 cães e adenocarcinoma em dois. Dois casos recidivaram a condição de hipercortisolemia, associada à hiperplasia, na adrenal contralateral, confirmados pelo teste de estimulação por ACTH. Concluiu-se que a adrenalectomia é um procedimento seguro, com poucas complicações perioperatórias, ressalvando-se a possiblidade de recidiva do quadro de hipercortisolismo.(AU)

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.

Mielolipoma adrenal gigante: Adrenalectomía derecha laparoscópica / Giant adrenal myelolipoma. Right laparoscopic adrenalectomy

El mielolipoma es un tumor adrenal poco frecuente. Se trata de un tumor benigno no funcionante compuesto por tejido adiposo y hematopoyético. Presentamos el caso de una paciente de 33 años de edad con diagnóstico de incidentaloma adrenal de 14 cm de diámetro no funcionante. Los estudios imagenológicos presentaban características sugestivas de mielolipoma. Debido a la naturaleza benigna del tumor se decidió el abordaje laparoscópico. La anatomía patológica informó un mielolipoma de 444 gramos. La paciente evolucionó favorablemente en el postoperatorio. El abordaje laparoscópico para la resección del mielolipoma gigante fue factible y exitoso.

Adrenal myelolipoma is a rare, benign, non-functioning tumor. It is composed by fat and hematopoietic tissues. We present the case of a 33-year-old woman with diagnosis of a 14 cm diameter non-functioning right adrenal incidentaloma, with imaging features suggestive of myelolipoma. Based on the benign nature of the tumor, laparoscopic resection was performed. Histopathology showed a myelolipoma, weighting 444 grams. The patient evolved with an uneventful postoperative period. Laparoscopic adrenalectomy for a giant myelolipoma was feasible and successful.