Enfermedad de Cushing recurrente con hipercortisolismo catastrófico: reporte de caso y revisión de literatura / Catastrophic hypercortisolism and recurrence in Cushing´s disease: a case report and literature review / Doença de Cushing recorrente com hipercortisolismo catastrófico: relato de caso e revisão da literatura

Resumen: Presentamos un caso de una paciente femenina de 27 años, con síndrome de Cushing ACTH dependiente con hipercortisolismo severo, causado por un macroadenoma hipofisario recurrente y resistente pese a dos cirugías transesfenoidales, radioterapia y terapia médica. Dada la falla en las diferentes terapias se realiza una adrenalectomía bilateral como tratamiento definitivo. La paciente fallece en el posoperatorio por causa no clara. Si bien la adrenalectomía bilateral ha sido reportada como un tratamiento efectivo en pacientes con enfermedad de Cushing, se ha relacionado con una mortalidad significativa vinculada con la severidad del hipercortisolismo y las comorbilidades presentes. En este caso la adrenalectomía izquierda se tuvo que convertir a cielo abierto, asociada con mayor morbimortalidad.

Abstract: The study presents the case of a 27-year-old female patient with adrenocorticotropic hormone (ACTH) dependent Cushing's disease and severe hypercortisolism caused by recurrent pituitary macroadenoma that was resistant to treatment despite two transsphenoidal surgeries, radiotherapy and medical treatment. Upon failure of the different therapies a bilateral adrenalectomy was performed as the final treatment. The patient died in after surgery although the case of death was not clear. Despite bilateral adrenalectomy having been reports as an effective treatment in patients with Cushing's disease, it has been related to significant mortality rates in connection with the severity of hypercortisolism and existing comorbilities. In this case the left adrenalectomy ended up being an open surgery, which is associated to a higher mortality rate.

Resumo: Apresentamos o caso de uma paciente de 27 anos com síndrome de Cushing ACTH-dependente com hipercortisolismo grave causado por macroadenoma hipofisário, recorrente e resistente, apesar de haver sido submetida a duas cirurgias transesfenoidal, radioterapia e terapia medicamentosa. Diante do fracasso das diferentes terapias, foi realizada adrenalectomia bilateral como tratamento definitivo. A paciente faleceu no pós-operatório por causa não esclarecida. Embora a adrenalectomia bilateral tenha sido relatada como tratamento eficaz em pacientes com doença de Cushing, ela tem sido associada a mortalidade significativa relacionada à gravidade do hipercortisolismo e às comorbidades presentes. Neste caso, a adrenalectomia esquerda teve que ser convertida para cirurgia aberta, associada a maior morbimortalidade.

Adrenalectomies in children and adolescents in Germany - a diagnose related groups based analysis from 2009-2017.

Background: Adrenalectomies are rare procedures especially in childhood. So far, no large cohort study on this topic has been published with data on to age distribution, operative procedures, hospital volume and operative outcome. Methods: This is a retrospective analysis of anonymized nationwide hospital billing data (DRG data, 2009-2017). All adrenal surgeries (defined by OPS codes) of patients between the age 0 and 21 years in Germany were included. Results: A total of 523 patient records were identified. The mean age was 8.6 ± 7.7 years and 262 patients were female (50.1%). The majority of patients were between 0 and 5 years old (52% overall), while 11.1% were between 6 and 11 and 38.8% older than 12 years. The most common diagnoses were malignant neoplasms of the adrenal gland (56%, mostly neuroblastoma) with the majority being younger than 5 years. Benign neoplasms in the adrenal gland (D350) account for 29% of all cases with the majority of affected patients being 12 years or older. 15% were not defined regarding tumor behavior. Overall complication rate was 27% with a clear higher complication rate in resection for malignant neoplasia of the adrenal gland. Bleeding occurrence and transfusions are the main complications, followed by the necessary of relaparotomy. There was an uneven patient distribution between hospital tertiles (low volume, medium and high volume tertile). While 164 patients received surgery in 85 different "low volume" hospitals (0.2 cases per hospital per year), 205 patients received surgery in 8 different "high volume" hospitals (2.8 cases per hospital per year; p<0.001). Patients in high volume centers were significant younger, had more extended resections and more often malignant neoplasia. In multivariable analysis younger age, extended resections and open procedures were independent predictors for occurrence of postoperative complications. Conclusion: Overall complication rate of adrenalectomies in the pediatric population in Germany is low, demonstrating good therapeutic quality. Our analysis revealed a very uneven distribution of patient volume among hospitals.

Minimally invasive adrenalectomy ­ Operative and perioperative results of transperitoneal and retroperitoneal adrenalectomies performed at the University of Szeged Department of Surgery during 23 years / Minimálisan invazív adrenalectomia - 23 év alatt végzett transperitonealis és retroperitonealis adrenalectomiák operatív és perioperatív eredményei a Szegedi Tudományegyetem Sebészeti Klinikán

Aim. Our goal was to evaluate operative and perioperative data of retroperitoneal (RP) and transperitoneal (TP) adrenalectomies performed at the University of Szeged Department of Surgery. Patients and method. During a retrospective cohort study including 174 adrenalectomies (28 RP; 146 TP) performed between 1998 and 2021, the following parameters were evaluated: rate of previous abdominal surgeries, conversion rate, operative time, intraoperative blood loss, tumor size, histology, hospital stay, early and late complications. Results. With significantly higher rate of previous abdominal surgeries [TP vs RP: 68 (46.57%) vs 4 (14%) P = 0.0021], there was no markable difference in conversion rate [TP vs RP: 7 (4.79%) vs 5 (18%), P = 0.312]. Significantly larger tumours were removed with TP (TP vs RP: 58.05 vs 34.8 mm, P = 0.016), with no markable difference in intraoperative blood loss (TP vs RP: 67.85 vs 50.2 ml, P = 0.157). Operative time was significantly shorter in TP (TP vs RP: 86.3 vs 134.5 min; P = 0.024). The most frequent histology was adenoma (TP vs RP: n = 95; 65.06% vs 64.3%). Pheochromocytoma occurred in 11 (7.53%) and 5 (17.8%) cases in TP and RP, respectively. We found no significant difference in hospital stay (TP vs RP: 5.125 vs 4.61 day; P = 0.413). Five- and 2 cases of early complications were seen in TP (splenic injury, postoperative fever, severe intraoperative bleeding, severe hypokalemia, surgical site infection) and RP (2 severe intraoperative bleeding), respectively. One lethal case of ventricular fibrillation and one delayed complication (postoperative abdominal wall hernia) were observed in TP. Conclusions. Both TP and RP are safe and simply reproducible minimally invasive techniques. According to our observation, RP adrenalectomy seems to be reserved for smaller lesions, while TP proves to be successful in removing enlarged and also malignant lesions with significantly shorter operative time.

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

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

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

Anaesthetic management of a dopamine-secreting phaeochromocytoma.

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

Outcomes after laparoscopic adrenalectomy for unilateral primary aldosteronism / Resultados luego de adrenalectomía laparoscópica en hiperaldosteronismo primario unilateral

Abstract Unilateral primary aldosteronism (PA) is the most common surgically correctable cause of hypertension. Determination of success after laparoscopic adrenalectomy (LA) is limited by the lack of standardized criteria. We sought to evaluate the surgical recurrence and functional outcomes of LA in patients with Conn's syndrome applying the primary aldosteronism surgical outcome (PASO) Criteria. Descriptive obser vational analysis of patients treated with LA due to confirmed u nilateral Conn's syndrome between May 2007 and August 2020: Twenty patients were included in the cohort; 16 patients had TLA and other four PLA [58% male, median age 47 (IQR: 44-59.5) years and median follow-up of 64 (IQR: 2-156) ] months. Median tumor size was 1.2 (0.8-1.8) cm. No conversions to open surgery were recorded and the overall morbidity of the series was 1/20. No surgical or biochemical recurrence was observed. Five patients were excluded from the analysis of functional results due to lack of follow-up. According to the PASO criteria, complete, partial, and no success were observed in 8/15, 6/15, and 1/15, respectively. The surgical treatment of the disease is supported by the literature, and we were able to reproduce the results of other series. The use of standardized and reproducible criteria to assess its functional results would be essential for a more complete and integrated evaluation of adrenal surgery.

Resumen El hiperaldosteronismo primario es la causa más frecuente de hipertensión secundaria pasible de tratamiento quirúrgico. La determinación del éxito de la adrenalectomía laparoscópica (AL), actualmente, está limitada por la falta de criterios estandarizados. Buscamos evaluar la tasa de recurrencia quirúrgica y los resultados funcionales de la AL en pacientes con Síndrome de Conn aplicando los criterios PASO (primary aldosteronism surgical outcome). Análisis descriptivo y observacional de pacientes tratados con AL en contexto de síndrome de Conn unilateral confirmado, entre Mayo-2007 y Agosto-2020. Se incluyeron 20 pacientes en el estudio; 16 pacientes tratados mediante AL total y 4 con AL parcial (55% hombres, edad mediana de 47 (IQR: 44-59.5) años y mediana de seguimiento 64 (IQR: 2-156) meses. La mediana de tamaño tumoral fue de 1.2 (0.8-1.8) cm. No se registraron conversiones a cirugía abierta y la morbilidad global de la serie: 1/20. No se observó recurrencia quirúrgica o bioquímica. Se excluyeron 5 pacientes en el análisis de resultados funcionales por falta de seguimiento. Según los criterios PASO, se observó un éxito completo, parcial y ausente en 8/15, 6/15 y 1/15, respectivamente. El tratamiento quirúrgico de la enfermedad es avalado por la literatura y pudimos reproducir los resultados de otras series. El uso estandarizado y reproducible de criterios para valorar sus resul tados funcionales sería fundamental para una evaluación más completa e integrada de la cirugía suprarrenal.

Adrenalectomía laparoscópica bilateral sincrónica: técnica quirúrgica y resultados perioperatorios en 13 años de experiencia / Synchronous bilateral laparoscopic adrenalectomy: Surgical technique and perioperative results of a 13-years’ experience

Introducción y objetivos: La cirugía laparoscópica se ha convertido en el abordaje estándar para el tratamiento de las glándulas suprarrenales. Debido a que la adrenalectomía bilateral sincrónica no se realiza con frecuencia, la evidencia sobre este procedimiento es limitada. Nuestro objetivo es reportar nuestra experiencia de 13 años con la adrenalectomía bilateral laparoscópica sincrónica, evaluando su viabilidad, seguridad y resultados perioperatorios.Pacientes y métodos: Un total de 23 pacientes consecutivos sometidos a adrenalectomía laparoscópica bilateral sincrónica entre 2007 y 2020 en un único centro académico fueron incluidos en el estudio. Las variables evaluadas fueron el tiempo quirúrgico, la pérdida media estimada de sangre, la conversión a cirugía abierta, las complicaciones postoperatorias, la mortalidad y la duración de la estancia postoperatoria.Resultados: El tiempo operatorio medio fue de 189,3±48,9min. La media de pérdida de sangre estimada fue de 163,0±201,3ml. No hubo conversiones a cirugía abierta. Cinco pacientes tuvieron complicaciones postoperatorias y 3 de estas fueron graves. Ningún paciente falleció durante el periodo perioperatorio. La mediana del tiempo de estancia postoperatoria fue de 3 días (rango 1-30). En el análisis patológico 15 pacientes tenían hiperplasia suprarrenal bilateral, 2 hiperplasia suprarrenal unilateral y un tumor benigno contralateral, uno hiperplasia suprarrenal unilateral y glándula contralateral normal, otro adenoma unilateral, 3 feocromocitomas bilaterales y uno mielolipoma bilateral.Conclusión: La adrenalectomía laparoscópica bilateral sincrónica es una técnica factible y segura. Se requiere un equipo multidisciplinar y experimentado que incluya anestesistas y endocrinólogos. (AU)

Introduction and objectives: Laparoscopic surgery is the standard approach for the treatment of adrenal glands. Bilateral synchronous adrenalectomy is rarely performed, and evidence about this procedure is limited. Our objective is to report our 13-year experience with synchronous laparoscopic bilateral adrenalectomy, evaluating its feasibility, safety, and perioperative outcomes.Patients and methods: A total of 23 consecutive patients undergoing synchronous bilateral laparoscopic adrenalectomy between 2007 and 2020 in a single academic center were included. Variables evaluated were operative time, estimated blood loss, conversion to open surgery, postoperative complications, mortality, and postoperative length of stay.Results: Mean operative time was 189.3±48.9min. Mean estimated blood loss was 163.0±201.3ml. There were no conversions to open surgery. Five patients had postoperative complications, three of those were major. No patient died in the perioperative period. Median postoperative length of stay was three days (range 1-30). At pathology analysis, 15 patients had bilateral adrenal hyperplasia, 2 unilateral adrenal hyperplasia and a contralateral benign tumor, 1 unilateral adrenal hyperplasia and a normal contralateral gland, 1 unilateral adenoma, 3 bilateral pheochromocytomas and 1 bilateral myelolipoma.Conclusion: Synchronous bilateral laparoscopic adrenalectomy is a feasible and safe technique. A multidisciplinary and experienced team involving anesthesiologists and endocrinologists is required. (AU)

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

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

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

Synchronous bilateral laparoscopic adrenalectomy: Surgical technique and perioperative results of a 13-years' experience.

INTRODUCTION AND OBJECTIVES: Laparoscopic surgery is the standard approach for the treatment of adrenal glands. Bilateral synchronous adrenalectomy is rarely performed, and evidence about this procedure is limited. Our objective is to report our 13-year experience with synchronous laparoscopic bilateral adrenalectomy, evaluating its feasibility, safety, and perioperative outcomes. PATIENTS AND METHODS: A total of 23 consecutive patients undergoing synchronous bilateral laparoscopic adrenalectomy between 2007 and 2020 in a single academic center were included. Variables evaluated were operative time, estimated blood loss, conversion to open surgery, postoperative complications, mortality, and postoperative length of stay. RESULTS: Mean operative time was 189.3 ±â€¯48.9 min. Mean estimated blood loss was 163.0 ±â€¯201.3 mL. There were no conversions to open surgery. Five patients had postoperative complications, three of those were major. No patient died in the perioperative period. Median postoperative length of stay was three days (range 1-30). At pathology analysis, 15 patients had bilateral adrenal hyperplasia, 2 unilateral adrenal hyperplasia and a contralateral benign tumor, 1 unilateral adrenal hyperplasia and a normal contralateral gland, 1 unilateral adenoma, 3 bilateral pheochromocytomas and 1 bilateral myelolipoma. CONCLUSION: Synchronous bilateral laparoscopic adrenalectomy is a feasible and safe technique. A multidisciplinary and experienced team involving anesthesiologists and endocrinologists is required.

Analysis of adrenalectomy for the treatment of adrenal diseases performed by the Public Health Service in São Paulo between 2008 and 2019 / Análise da adrenalectomia para tratamento de doenças adrenais realizadas pelo Serviço Público de Saúde de São Paulo entre 2008 e 2019

ABSTRACT Introduction: treating benign (hormonally active or nonfunctional) and malignant adrenal cancer includes adrenalectomy. The expertise of surgeons and surgery performed by high-volume surgeons were associated with fewer complications and lower cost. We aimed to describe and compare the number of surgeries, mortality rate, and length of hospital stay for adrenalectomies performed between 2008 and 2019 in the public health system of São Paulo. Methods: this was an ecological study. The data were collected using the TabNet Platform of the Unified Health System Department of Informatics. Outcomes analyzed included the number of surgeries performed, mortality rate during hospital stay, and length of hospital stay. Public hospitals in Sao Paulo were divided into three subgroups according to the surgical volume of adrenalectomies performed as well as hospitals with and without a residency program in Urology, and the results were compared among them. Results: a total of 943 adrenalectomies were performed in Sao Paulo between 2008 and 2019. Mortality rates during hospital stay according to hospital surgical volume were no reported deaths in low-volume, 0.015% in intermediate-volume, and 0.004% in high-volume hospitals. The average length of the ICU stay was 1.03 days in low-volume, 2.8 in intermediate-volume, and 1.12 in high-volume hospitals (analysis between intermediate and high volume centers with statistical significance, p=0.016). Conclusions: despite no statistically significant differences among the groups analyzed, mortality rates were very low in all groups. ICU stay was shorter in high-volume centers than in intermediate-volume centers.

RESUMO Introdução: o tratamento do câncer de adrenal benigno (hormonalmente ativo ou não funcional) e maligno inclui a adrenalectomia. A experiência dos cirurgiões e a cirurgia realizada por cirurgiões de alto volume foram associadas a menos complicações e menor custo. O objetivo do estudo foi descrever e comparar o número de cirurgias, a taxa de mortalidade e o tempo de internação para adrenalectomias realizadas entre 2008 e 2019 na rede pública de saúde de São Paulo. Métodos: trata-se de um estudo ecológico. Os dados foram coletados da Plataforma TabNet do Departamento de Informática do Sistema Único de Saúde. Os hospitais foram divididos em três subgrupos de acordo com o volume cirúrgico e hospitais com e sem programa de residência médica em Urologia. Os resultados foram comparados entre os grupos. Resultados: no período estudado, 943 adrenalectomias foram realizadas em São Paulo. As taxas de mortalidade durante a internação de acordo com o volume cirúrgico hospitalar foram: não foram relatados óbitos em hospitais de baixo volume; 0,015% em hospitais de volume intermediário e 0,004% em alto volume. O tempo médio de permanência na UTI foi de 1,03 dias nos hospitais de baixo volume; 2,8 nos de médio volume e 1,12 nos de alto volume (análise entre centros de médio e alto volume com significância estatística, p=0,016). Conclusão: as taxas de mortalidade foram muito baixas em todos os grupos. A permanência na UTI foi menor em centros de alto volume do que em centros de volume intermediário.

Outcomes following surgical management of adrenocortical carcinoma: A single-center experience. / Resultados del manejo quirúrgico del carcinoma adrenocortical: experiencia de un centro hospitalario.

OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare and heterogeneous disease, with challenging management and poor prognosis. Surgery withcurative intent is the preferred treatment option for localized disease, with a reported 5-year survival rate of 55% for complete resections. However, owing to the high risk of recurrence there is a need for adjuvant therapies, such as mitotane, an adrenolytic drug, or irradiation, while in advanced disease the standard of careis a combined chemotherapy scheme. The aim of this study was to report our experience in the surgical management and outcomes of ACC patients. MATERIALS AND METHODS: A retrospective observational study was performed in a cohort of ACC patients who had undergone surgical resection (open or laparoscopic approach) and were followed up at our tertiary hospital. Patients with localized or locally advanced disease were included in the analysis. All medical records, including clinical, surgical, pathologic, and follow-updata, were collected and analyzed. RESULTS: A total of 19 ACC patients were managed at our center between August 1990 and August 2013. The median age at diagnosis was 50.5 years (range 19-72), and most patients were males. Abdominal pain was the most common clinical presentation (n=9,47.4%). Abdominal contrast-enhanced computed tomography (CT) was performed in all cases. Only 3 tumors (15.8%) were functional and most were stage II at diagnosis (n=9, 47.4%). No patient presented metastasis. Of the 19 patients, 18 (94.7%) under went surgerywith curative intent, while one (5.3%) received adjuvant radiotherapy (ART). The open approach was used in 17 patients (89.5%), while the remaining 2 (10.5%) underwent laparoscopy. Postoperative complications occurred in 8 patients (42.1%); none were of grade IV or V. Median follow-up was 66 months (range 3-312). The majority of patients (n=15, 78.9%) were disease free with surgery alone. None received adjuvant mitotane therapy (AMT). Four patients (21%) experienced metachronous metastases and 3 (15.8%) local recurrence after a median time of 10.5 months (range 2-60) and 9.3 months (range 1.5-30), respectively. The 5-year overall survival rate was 47.4%. CONCLUSIONS: Our findings confirm both the unpredictable nature of ACC and the accepted primary role of surgery. The use of adjuvant therapy was less frequent in this series than is supported currently. However, a multidiscipinary approach should be the initial step in the management of this rare malignancy.

OBJETIVO: El carcinoma adrenocortical es una enfermedad heterogénea y rara que conlleva un manejo complicado y cuyo pronóstico es malo. Ante una enefermedad localizada el manejo quirúrgico es de elección, observando una tasa de supervivencia a los 5 años del 55% en resecciones completas. Sin embargo, debido a la alta tasa de recurrencias, se requiere complementar el manejo con una terapia adyuvante como es el caso del mitotane, un agente adrenolítico, o la radioterapia. En casos de enfermedad avanzada la terapia de elección incluye una pauta de quimioterapia combinada. El objetivo del presente estudio es reportar nuestra experiencia en el manejo quirúrgico y resultados obtenidos de los pacientes con diagnóstico de carcinoma adrenocortical. MATERIALES Y MÉTODOS: Se realizó un estudio observacional de una cohorte de pacientes diagnosticados de carconinoma adrenocortical que requirieron un manejo quirúrgico (abierto o laparoscópico) y que realizaron su seguimiento en nuestro hospital terciario. Se incluyó a pacientes con enfermedad localizada y localmente avanzada en el análisis. Todos los dados clínicos, quirúrgicos, patológicos y de seguimiento fueron recolectados para el análisis. RESULTADOS: Entre agosto del 1990 y agosto del 2013 un total de 19 pacientes diagnosticados de carcinoma adrenocortical fueron tratados en nuestro centro. La mediana de edad al diagnóstico fue 50,5 años (rango 19-72), siendo la mayoría hombres. El síntoma inicial mas frecuente fue el dolor abdominal (n=9, 47,4%). Una tomografía computarizada con contraste fue realizada en todos los casos. Sólo 3 tumores (15,8%) eran funcionales y la mayoría correspondía a un estadio II al diagnóstico (n=9, 47,4%). Ningún paciente presentaba metástasis. De los 19 pacientes,18 (94,7%) requirieron cirugía con intención curativa, mientras que a uno (5,3%) se le realizó radioterapia adyuvante. El abordaje abierto se usó en 17 pacientes (89,5%), mientras que a 2 pacientes (10,5%) se les realizó una técnica laparoscópica. Complicaciones postquirúrgicas ocurrieron en 8 pacientes (42,1%), de las cuales ninguna fue de grado IV o V. La mediana de seguimiento fue de 66 meses (rango 3-312). La mayoría de los pacientes (n=15, 78,9%) permanecieron libres de enfermedad únicamente con un manejo quirúrgico. Ninguno recibió terapia adyuvante con mitotane. Cuatro pacientes (21%) presentaron metástasis metacrónicas y 3 (15,8%) recurrencia local tras una mediana de tiempo de 10,5 meses (rango 2-60) y 9,3 meses (rango1,5-30), respectivamente. La tasa de supervivencia globala los 5 años fue del 47,4%. CONCLUSIONES: Nuestros resultados confirman tanto la naturaleza impredecible del carcinoma adrenocortical como la aceptación del rol primario de la cirugía en su manejo. El uso de un agente adyuvante fue menos frecuente en esta serie respecto a lo respaldado actualmente. Sin embargo, un manejo multidisciplinar debería ser el primer paso a seguir en el tratamiento de esta rara enfermedad.

Outcomes following surgical management of adrenocortical carcinoma: a single-center experience / Resultados después del manejo quirúrgico del carcinoma adrenocortical: una experiencia de un solo centro

Objetive: Adrenocortical carcinoma(ACC) is a rare and heterogeneous disease, with challenging management and poor prognosis. Surgery withcurative intent is the preferred treatment option for localized disease, with a reported 5-year survival rate of55% for complete resections. However, owing to thehigh risk of recurrence there is a need for adjuvant therapies, such as mitotane, an adrenolytic drug, or irradiation, while in advanced disease the standard of careis a combined chemotherapy scheme. The aim of thisstudy was to report our experience in the surgical management and outcomes of ACC patients. Material and methods: A retrospective observational study was performed in a cohort of ACC patientswho had undergone surgical resection (open or laparoscopic approach) and were followed up at our tertiary hospital. Patients with localized or locally advanced disease were included in the analysis. All medical records,including clinical, surgical, pathologic, and follow-updata, were collected and analyzed. Results: A total of 19 ACC patients were managedat our center between August 1990 and August 2013.The median age at diagnosis was 50.5 years (range19–72), and most patients were males. Abdominalpain was the most common clinical presentation (n=9,47.4%). Abdominal contrast-enhanced computed tomography (CT) was performed in all cases. Only 3 tu-mors (15.8%) were functional and most were stage II atdiagnosis (n=9, 47.4%). No patient presented metastasis. Of the 19 patients, 18 (94.7%) underwent surgerywith curative intent, while one (5.3%) received adjuvantradiotherapy (ART). The open approach was used in17 patients (89.5%), while the remaining 2 (10.5%)underwent laparoscopy. Postoperative complicationsoccurred in 8 patients (42.1%); none were of grade IVor V. Median follow-up was 66 months (range 3–312).The majority of patients (n=15, 78.9%) were diseasefree with surgery alone. None received adjuvant mitotane therapy...(AU)

Objetivo: El carcinoma adrenocorticales una enfermedad heterogénea y rara que conllevaun manejo complicado y cuyo pronóstico es malo. Anteuna enefermedad localizada el manejo quirúrgico es deelección, observando una tasa de supervivencia a los 5años del 55% en resecciones completas. Sin embargo,debido a la alta tasa de recurrencias, se requiere complementar el manejo con una terapia adyuvante comoes el caso del mitotane, un agente adrenolítico, o laradioterapia. En casos de enfermedad avanzada la terapia de elección incluye una pauta de quimioterapiacombinada. El objetivo del presente estudio es reportarnuestra experiencia en el manejo quirúrgico y resultadosobtenidos de los pacientes con diagnóstico de carcinoma adrenocortical. Materiales y métodos: Se realizó un estudio observacional de una cohorte de pacientes diagnosticados de carconinoma adrenocortical que requirieronun manejo quirúrgico (abierto o laparoscópico) y querealizaron su seguimiento en nuestro hospital terciario.Se incluyó a pacientes con enfermedad localizada ylocalmente avanzada en el análisis. Todos los dados clínicos, quirúrgicos, patológicos y de seguimiento fueronrecolectados para el análisis. Resultados: Entre agosto del 1990 y agosto del2013 un total de 19 pacientes diagnosticados decarcinoma adrenocortical fueron tratados en nuestrocentro. La mediana de edad al diagnóstico fue 50,5años (rango 19-72), siendo la mayoría hombres. Elsíntoma inicial mas frecuente fue el dolor abdominal(n=9, 47,4%). Una tomografía computarizada con contraste fue realizada en todos los casos. Sólo 3 tumores(15,8%) eran funcionales y la mayoría correspondíaa un estadio II al diagnóstico (n=9, 47,4%). Ningúnpaciente presentaba metástasis. De los 19 pacientes,18 (94,7%) requirieron cirugía con intención curativa,mientras que a uno (5,3%) se le realizó radioterapiaadyuvante...(AU)

Rol actual de la adrenalectomía bilateral en el manejo del síndrome de cushing ectópico / Current role of bilateral adrenalectomy in the management of ectopic Cushing's syndrome

Objetivo: Describir las característicasclínicas y los resultados perioperatorios de la adrenalectomía bilateral laparoscópica (ABL) en 5 pacientestratados con diagnóstico de Síndrome de Cushing Ectópico (SCE).Material y método: Se realizó un estudio retrospectivo y descriptivo de todos los pacientes con SCEsometidos a una ABL, entre enero de 2009 y junio de2017. Todos los pacientes fueron evaluados por unequipo multidisciplinario que decidió conjuntamente elmomento más adecuado para la cirugía. Fueron analizados los siguientes datos protocolares: edad, sexo, origen del tumor secretor, duración del hipercortisolismo,motivo de internación, manejo terapéutico inicial, datosbioquímicos (cortisol sérico preoperatorio, ACTH plasmática y cortisol libre urinario), datos quirúrgicos (vía deabordaje, tiempo quirúrgico, tipo de cirugía, necesidadde conversión a cirugía abierta), datos perioperatorios (tiempo de internación postoperatoria, complicacionessegún la escala de Clavien-Dindo, mortalidad, tasa decuración clínica y tasa de curación bioquímica, tiempode seguimiento).Resultados: Cinco pacientes fueron sometidos a ABL.El abordaje fue mínimamente invasivo, ya sea transperitoneal (n=3) o retroperitoneoscópico posterior (n=2). Eltiempo de internación fue de 23,4 días (r=4-81). Ningún paciente falleció por complicaciones quirúrgicas, ytodos lograron la curación bioquímica y clínica de suhipercortisolismo después de la cirugía.Conclusiones: ABL es un procedimiento de rescateeficaz para controlar los síntomas asociados al hipercortisolismo en pacientes con SCE refractario al tratamiento médico.(AU)

Objectives: The aim of this paper is todescribe the clinical features and the perioperative results of the bilateral laparoscopic adrenalectomy (BLA) ofsalvaje in 5 patients treated for an Ectopic Cushing Syndrome (CS) who were refractory to medical treatment.Material and methods: We performed a retrospective analysis of the clinical records of our institution.Five BLAs were performed in patients with Ectopic CSrefractory to medical treatment during the period fromJanuary 2009 and June 2017. All patients were evaluated by a multidisciplinary team that jointly decidedthe most appropriate time for surgery. The followingprotocol data were analyzed: age, sex, location of the secreting tumor, duration of hypercortisolism, reason forhospitalization, initial therapeutic management), biochemical data (pre-op serum cortisol, plasma ACTH andfree urinary cortisol), surgical data (surgical approach,surgical time, type of surgery, need for conversion toopen surgery), perioperative data (post op surgical stay,post op complications according to the Clavien-Dindoscale 14, mortality, clinical cure rate and biochemicalcure rate, follow up time).Results: Five patients underwent BLA. The approachfor the BLA was minimally invasive, either trans peritoneal (n=3) or posterior retroperitoneoscopic (n=2). Meanhospitalization time was 23.4 days (r=4-81). None ofthe patients died because of surgical complications, andall of them achieved biochemical and clinical remissionof their hypercortisolism after surgery.Conclusions: BLA is an effective salvage alternativeto control the symptoms associated with overproductionof corticosteroids in patients with ectopic CS refractoryto medical treatment.(AU)

[Current role of bilateral adrenalectomy in Ectopic Cushing syndrome.] / Rol actual de la adrenalectomía bilateral en el manejo del síndrome de Cushing Ectópico.

OBJECTIVES: The aim of this paper is to describe the clinical features and the perioperative results of the bilateral laparoscopic adrenalectomy (BLA) of salvaje in 5 patients treated for an Ectopic Cushing Syndrome (CS) who were refractory to medical treatment. MATERIALS AND METHODS: We performed a retrospective analysis of the clinical records of our institution. Five BLAs were performed in patients with Ectopic CS refractory to medical treatment during the period from January 2009 and June 2017. All patients were evaluated by a multidisciplinary team that jointly decided the most appropriate time for surgery. The following protocol data were analyzed: age, sex, location of the secreting tumor, duration of hypercortisolism, reason for hospitalization, initial therapeutic management), biochemical data (pre-op serum cortisol, plasma ACTH and free urinary cortisol), surgical data (surgical approach, surgical time, type of surgery, need for conversion toopen surgery), perioperative data (post op surgical stay, post op complications according to the Clavien-Dindo scale 14, mortality, clinical cure rate and biochemical cure rate, follow up time). RESULTS: Five patients underwent BLA. The approach for the BLA was minimally invasive, either trans peritoneal (n=3) or posterior retroperitoneoscopic (n=2). Mean hospitalization time was 23.4 days (r=4-81). None of the patients died because of surgical complications, and all of them achieved biochemical and clinical remission of their hypercortisolism after surgery. CONCLUSIONS: BLA is an effective salvage alter native to control the symptoms associated with overproduction of corticosteroids in patients with ectopic CS refractory to medical treatment.

OBJETIVO: Describir las características clínicas y los resultados perioperatorios de la adrenalectomía bilateral laparoscópica (ABL) en 5 pacientes tratados con diagnóstico de Síndrome de Cushing Ectópico (SCE).MATERIALES Y MÉTODOS: Se realizó un estudio retrospectivo y descriptivo de todos los pacientes con SCE sometidos a una ABL, entre enero de 2009 y junio de 2017. Todos los pacientes fueron evaluados por un equipo multidisciplinario que decidió conjuntamente el momento más adecuado para la cirugía. Fueron analizados los siguientes datos protocolares: edad, sexo, origen del tumor secretor, duración del hipercortisolismo, motivo de internación, manejo terapéutico inicial, datos bioquímicos (cortisol sérico preoperatorio, ACTH plasmática y cortisol libre urinario), datos quirúrgicos (vía de abordaje, tiempo quirúrgico, tipo de cirugía, necesidad de conversión a cirugía abierta), datos perioperatorios (tiempo de internación postoperatoria, complicaciones según la escala de Clavien-Dindo, mortalidad, tasa de curación clínica y tasa de curación bioquímica, tiempo de seguimiento). RESULTADOS: Cinco pacientes fueron sometidos a ABL. El abordaje fue mínimamente invasivo, ya sea transperitoneal (n=3) o retroperitoneoscópico posterior (n=2). El tiempo de internación fue de 23,4 días (r=4-81). Ningún paciente falleció por complicaciones quirúrgicas, y todos lograron la curación bioquímica y clínica de su hipercortisolismo después de la cirugía. CONCLUSIONES: ABL es un procedimiento de rescate eficaz para controlar los síntomas asociados al hipercortisolismo en pacientes con SCE refractario al tratamiento médico.

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

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

Neoplasia endocrina múltiple IIA: feocromocitoma bilateral. A propósito de un caso y revisión de la literatura / Multiple Endocrine Neoplasia IIA: Bilateral Pheochromocytoma. About a Case and Literature Review

Resumen: Presentamos el caso de una paciente del sexo femenino, de 54 años de edad, con antecedente familiar de neoplasia endocrina múltiple tipo II, con estudio genético positivo para NEM 2 IIA EXON 11, c634 CGCIB. Inició padecimiento con cardiopatía hipertensiva y disfunción diastólica, hallazgo to mográfico de tumor suprarrenal bilateral por imagenología, se realizó adrenalectomía convencional transabdominal, se encontró tumor derecho con cápsula correspondiente a feo cromocitoma con un peso de 1,100 g de 14.5 cm de diámetro mayor con invasión a cápsula sin romperla, tumor suprarrenal izquierdo correspondiente a feocromocitoma con un peso de 950 g de 15 cm de diámetro mayor.

Abstract: We present the case of a 54-year-old female patient with a family history of multiple endocrine neoplasia type II, with a genetic study for MEN 2 IIA EXON 11, CG6B c634, onset with hypertensive heart disease and diastolic dysfunction, tomographic finding of bilateral adrenal tumor by imaging. A conventional transabdominal adrenalectomy was performed, finding a right tumor with a capsule corresponding to pheochromocytoma with a weight of 1,100 g of 14.5 cm of greater diameter with invasion of the capsule without breaking it, and a left adrenal tumor corresponding to pheochromocytoma with a weight of 950 g of 15 cm of greater diameter.

Clevidipine as an antihypertensive drug in three pheochromocytoma surgeries: Case series / Clevidipino como fármaco antihipertensivo en tres cirugías de feocromocitoma: Serie de casos

Abstract Introduction Laparoscopic adrenalectomy is the treatment of choice of pheochromocytoma. During the first surgical phase (pneumoperitoneum insufflation, tumor and veins handling), there is a risk of hypertensive crisis due to catecholamine release. After tumor excision, patients can suffer relative vasodilation and the residual effect of antihypertensive drugs, which results in arterial hypotension. For that reason, antihypertensive drugs used in the first phase should have a rapid onset of action, short half-life and no residual effect. Methods We report a series of three cases of patients with pheochromocytoma who were treated with laparoscopic adrenalectomy. They all received clevidipine infusion from the beginning of the surgery, before they had presented hypertension, to treat and try to minimize hypertensive peaks. Results In all patients, hypertensive peaks were controlled in a few minutes. After tumor resection, clevidipine infusion was stopped in all cases, and any patient required infusion of vasopressors. Discussion Clevidipine could be a first choice antihypertensive drug in pheochromocytoma surgery. Starting the infusion of clevidipine before the hypertensive peaks could help to make them less pronounced.

Resumen Introducción La adrenalectomía laparoscópica es el tratamiento de elección del feocromocitoma. Durante la primera fase quirúrgica (insuflación de neumoperitoneo, manipulación del tumor y de las venas implicadas), existe el riesgo de que se desencadenen crisis hipertensivas debido a la liberación de catecolaminas. Después de la extirpación del tumor, los pacientes pueden sufrir una vasodilatación relativa y el efecto residual de los fármacos antihipertensivos usados previamente, lo que resulta en hipotensión arterial. Por esa razón, los fármacos antihipertensivos utilizados en la primera fase quirúrgica deben tener rápido inicio de acción, vida media corta y mínimo efecto residual. Métodos Se describe una serie de casos de tres pacientes con feocromocitoma que fueron tratados con adrenalectomía laparoscópica. Todos recibieron infusión de clevidipino desde el comienzo de la cirugía, antes de presentar hipertensión arterial, para así intentar minimizar y tratar rápidamente los posibles picos hipertensivos. Resultados En todos los pacientes los picos hipertensivos se controlaron en pocos minutos. Después de la resección del tumor, la infusión de clevidipino se detuvo en todos los casos y ningún paciente requirió perfusión de vasopresores. Discusión El clevidipino podría ser un fármaco antihipertensivo de primera elección en la cirugía de feocromocitoma. Iniciarlo antes de que ocurran los picos hipertensivos podría ayudar a que sean más leves.

Ganglioneuroma suprarrenal gigante: Reporte de Un caso

Resumen El ganglioneuroma (Gn) es una neoplasia benigna, rara, derivada de la cresta neural, compuesta de células ganglionares maduras y células de Schwann. Solo una pequeña proporción de Gn se origina en la médula suprarrenal, siendo esta localización más frecuente en niños y adultos jóvenes. Hemos encontrado menos de 30 publicaciones reportadas en la literatura. Presentamos el caso de una paciente femenina de 37 años de edad, con dolor intermitente en fosa renal izquierda y flanco ipsilateral de 5 meses de evolución, sin otros síntomas acompañantes. Se realizó ecografía abdominal, tomografía axial computada (TAC) y resonancia magnética nuclear (RMI) abdomino pélvica, en las cuales se identificó formación expansiva solida, de 85 x 58 x 74 mm, de contornos definidos, adyacente a riñón izquierdo. Las características por imagen no permitieron descartar lesión benigna. Se realizó perfil hormonal completo para despistaje de funcionalidad. Dadas las características imagenológicas y el tamaño de la lesión, se decide tratamiento quirúrgico, considerando a feocromocitoma silente como principal diagnóstico presuntivo pre quirúrgico. La evolución postoperatoria fue excelente. Al examen microscópico, se observa proliferación nodular, de bordes delimitados, formado por células fusadas, con núcleos bipolares y citoplasmas elongados con aisladas células ganglionares. En la inmunomarcación presenta aisladas células ganglionares positivas para Cromogranina A y Sinaptofisina, proteína S100 positivo difuso, hallazgos consistentes con ganglioneuroma suprarrenal. El hallazgo de una masa suprarrenal en paciente joven, sin síntomas específicos, con características no sugestivas de adenoma, debe considerar el diagnostico de ganglioneuroma como parte de las lesiones incidentales adrenales y destacamos la importancia del diagnostico diferencial con feocromocitoma y carcinoma adrenal ya que comparten similares características imagenológicas. El tratamiento de elección es la resección quirúrgica completa, con pronóstico excelente en la mayoría de los casos.

Successful surgical treatment of a rare, large adrenal hemangioma / Ritka elofordulású, nagy méretu mellékvese-haemangioma sikeres sebészi kezelése.

Összefoglaló. A mellékvese-haemangiomák ritkán eloforduló, nehezen diagnosztizálható elváltozások, melyek sebészi eltávolítása gyakran nagy fokú technikai jártasságot igényel. Vizsgálatunkban egy 69 éves nobeteg esetét ismertetjük, akinél fogyás és hypertonia miatt végzett kivizsgálás mellékvesecisztát feltételezett, valamint felvetette adrenocorticalis carcinoma jelenlétét. A preoperatív kivizsgálás során (CT és MRI) a felmerülo malignitás miatt sebészi eltávolítás vált szükségessé. A kezdeti laparoszkópos transperitonealis technikát követoen a bonyolult elhelyezkedés, illetve vérzés miatt kis méretu, paramedián metszésbol konvertáltunk, és bal oldali nyitott adrenalectomiát végeztünk, valamint a vese caudalis részérol egy folyadéktartalmú képletet távolítottunk el. A szövettani feldolgozás a vese középso harmadának magasságából reszekált cisztát, valamint a cranialis lokalizációról eltávolított, ritka elofordulású mellékvese-haemangiomát igazolt. A ritkán eloforduló és többnyire bizonytalan preoperatív diagnózissal bíró mellékvese-haemangiomák sebészi eltávolítása nagy méretük, kompresszióra való hajlamuk, valamint malignitást utánzó megjelenésük miatt is indokolt. A laparoszkópos transperitonealis adrenalectomia extra nagy méretu (>10 cm ) és malignus tumorok, illetve nagy méretu haemangiomák eltávolítására is alkalmas eljárás. A mellékvese-haemangiomák sebészi reszekciója nagyfokú körültekintést igényel, megnövekedett vérzéshajlamuk, valamint a gyakori, nagyerekhez való közeli elhelyezkedés miatt a konverzió veszélye is jelentosen növekszik. Orv Heti. 2020; 161(47): 2006-2010. Summary. Adrenal hemangiomas are rare. Their preoperative diagnosis is usually vague, and often require advanced surgical skills for resection. We herein describe the case of a 69-year-old female patient initially presented with weight loss and hypertension. Preoperative Computed Tomography (CT) and magnetic resonance imaging (MRI) confirmed the presence of an adrenal cyst and possible adrenocortical carcinoma requiring surgical intervention. After initial laparoscopic transperitoneal approach, conversion to open surgery through a small paramedian incision was carried out due to the lesion's problematic location and continuous intraoperative bleeding. Open adrenalectomy along with the removal of a fluid-bearing lesion from the caudal pole of the kidney was performed. Histology confirmed a cyst removed from the mid-third, and a rare occurring adrenal hemangioma from the cranial part of the kidney. Adrenal hemangiomas usually bear uncertain preoperative diagnosis. Surgical removal becomes necessary in case of increasing size, potential to compress neighbouring structures and possible malignancy. Laparoscopic transperitoneal adrenalectomy is a feasible approach for the removal of extra large (>10 cm) and even malignant lesions as well as for large hemangiomas. Surgery of adrenal hemangiomas require a high level of caution, moreover, their potential for bleeding and frequent vicinity to nearby vascular structures may increase the need for open surgery. Orv Hetil. 2020; 161(47): 2006-2010.

Treatment of primary hyperaldosteronism. / Tratamiento del hiperaldosteronismo primario.

Primary aldosteronism is associated with higher cardiovascular and renal morbidity and mortality than essential hypertension in age- and sex-matched patients with the same degree of blood pressure elevation. Therefore, it is essential to establish a specific treatment to avoid the deleterious effects of aldosterone excess. Although adrenalectomy is generally considered the treatment of choice in cases of primary aldosteronism due to unilateral disease, several aspects and circumstances should be taken into account that may make medical treatment more appropriate. Among them, in this review we mention the limited experience and efficacy, and the potential risks of adrenal vein sampling; the risks and low efficacy of adrenalectomy; the high safety and efficacy of medical treatment and some special situations such as primary aldosteronism during pregnancy, in patients of advanced age or hereditary forms of primary aldosteronism, in which medical treatment is considered especially indicated as the first line therapy. The main studies comparing medical and surgical treatment in primary aldosteronism are also discussed.