Seventy years of pheochromocytomas and paragangliomas in Argentina. The FRENAR database.

Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors characterized by the excessive production of catecholamines. This study aims to describe the clinical characteristics of PPGL cases in Argentina over recent decades. A multicenter retrospective cross-sectional analysis was carried out using a database comprising both pediatric and adult patients with confirmed PPGL diagnoses based on pathological reports. A cohort of 486 patients with PPGL was recruited. Women represent 58.4% of the patients, with a mean age of 38.3 years old at the time of diagnosis and 15.2% of the patients were under the age of 18. Hypertension, as well as classic signs and symptoms, were present in 80.9% of the patients. The adrenal incidentaloma, as a mode of presentation, increased in the last two decades rising from 3.9% (1953-2000) to 21.8% (2001-2022), p<0.001. Most tumors were located within the adrenal glands, accounting 83.0% of the cases, with bilateral occurrences noted in 20.0%. The median tumor size was 4.8cm. Local recurrence and metastases were observed in 10.9% and 12.2%. Out of 412 patients, 87.0% exhibited urinary excretion elevation of catecholamines and/or their metabolites. Furthermore, 148 patients, representing 30.4% of the study population, displayed a distinct genetic profile indicative of hereditary syndromes. The distribution of hereditary syndromes revealed that MEN2, VHL, and PGL4 constituted the most prevalent syndromes. This population-based study, spanning seven decades, offers valuable insights into the demographic and clinical characteristics of PPGL patients in Argentina.

A not so incidental 'incidentaloma' - pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review.

Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental. Surgical resection led to a robust clinical improvement, and pathology revealed a benign ganglioneuroma. This report widens the spectrum of paraneoplastic manifestations of ganglioneuroma, reviews the diagnostic approach to antibody-negative pediatric AE, and raises important clinical considerations regarding benign and incidentally found tumors in the setting of a suspected paraneoplastic neurologic syndrome.

Evaluación del incidentaloma adrenal / Evaluation of the adrenal incidentaloma

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

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

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

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

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

Obesidad secundaria a síndrome endocrinológico en paciente pluripatológica / Obesity secondary to endocrinology syndrome in a polymorbid patient

Presentamos el caso de una paciente de 64 años, natural de Rumanía, con antecedentes médicos de síndrome de apnea-hipopnea, cirrosis hepática, EPOC y artrosis. Fumadora activa de un paquete de cigarrillos/día. La paciente es remitida desde la consulta de neumología para estudio ambulatorio por presentar como hallazgo incidental adenomas suprarrenales bilaterales y aumento progresivo de peso en los últimos 5 años. En el estudio de adenomas suprarrenales y obesidad se objetiva la presencia de un síndrome de Cushing. Es importante estudiar la aparición de la obesidad reciente en una paciente pluripatológica dado que puede haber otras causas secundarias además de las enfermedades subyacentes que presenta. Durante la presentación del caso clínico se realiza un abordaje esencial y práctico de la enfermedad adrenal. (AU)

We present the case of a 64-year-old woman from Romania with a medical history of obstructive sleep apnea-hypopnea syndrome, hepatic cirrhosis, COPD, and osteoarthritis. She smokes one pack of cigarettes per day. The patient is referred from the pulmonology department for an outpatient evaluation for an incidental finding of bilateral adrenal adenomas and progressive weight gain over the last five years. Cushing syndrome is detected during study of the adrenal adenomas and obesity. It is important to study recent-onset obesity in multimorbid patients as there could be other secondary causes in addition to the underlying diseases they present with. During the presentation of this clinical case, an essential, practical approach to the adrenal pathology is made. (AU)

Obesity secondary to endocrinology syndrome in a polymorbid patient.

We present the case of a 64-year-old woman from Romania with a medical history of obstructive sleep apnea-hypopnea syndrome, hepatic cirrhosis, COPD, and osteoarthritis. She smokes one pack of cigarettes per day. The patient is referred from the pulmonology department for an outpatient evaluation for an incidental finding of bilateral adrenal adenomas and progressive weight gain over the last five years. Cushing syndrome is detected during study of the adrenal adenomas and obesity. It is important to study recent-onset obesity in multimorbid patients as there could be other secondary causes in addition to the underlying diseases they present with. During the presentation of this clinical case, an essential, practical approach to the adrenal pathology is made.

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.

Functional imaging of adrenal cortex. / Estudios de imagen funcional de la corteza adrenal.

The rising number of high-resolution imaging scans has increased the adrenal lesions detection, which require a differential diagnosis. Currently, the most commonly used scans are CT and MRI, but these are sometimes not very specific. In these cases, nuclear medicine scans with 131I-norcolesterol, 11C-metomidate and 18F-fludeoxyglucose help to differentiate benign vs. malignant lesions, to lateralize the involvement in hypersecretion disease, as well as to guide the therapeutic strategy in both unilateral and bilateral lesions.

Practical guide on the initial evaluation, follow-up, and treatment of adrenal incidentalomas Adrenal Diseases Group of the Spanish Society of Endocrinology and Nutrition. / Guía práctica sobre la evaluación inicial, seguimiento y tratamiento de los incidentalomas adrenales. Grupo de patología adrenal de la Sociedad Española de Endocrinología y Nutrición.

Initial evaluation of adrenal incidentalomas should be aimed at ruling out malignancy and functionality. For this, a detailed clinical history should be taken, and an adequate radiographic assessment and a complete blood chemistry and hormone study should be performed. The most controversial condition, because of the lack of consensus in its definition, is autonomous cortisol secretion. Our recommendation is that, except when cortisol levels <1.8µg/dL in the dexamethasone suppression test rule out diagnosis and levels ≥5µg/dL establish the presence of autonomous cortisol secretion, diagnosis should be based on a combined definition of dexamethasone suppression test ≥3µg/dL and at least one of the following: elevated urinary free cortisol, ACTH level <10 pg/mL, or elevated nocturnal cortisol (in serum and/or saliva). During follow-up, dexamethasone suppression test should be repeated, usually every year, on an individual basis depending on the results of prior tests and the presence of comorbidities potentially related to hypercortisolism. The initial radiographic test of choice for characterization of adrenal incidentalomas is a computed tomography scan without contrast, but there is no unanimous agreement on subsequent monitoring. Our general recommendation is a repeat imaging test 6-12 months after diagnosis (based on the radiographic characteristics of the lesion). If the lesion remains stable and there are no indeterminate characteristics, no additional radiographic studies would be needed. We think that patients with autonomous cortisol secretion with comorbidities potentially related to hypercortisolism, particularly if they are young and there is a poor control, may benefit from unilateral adrenalectomy. The indication for unilateral adrenalectomy is clear in patients with overt hormonal syndromes or suspected malignancy. In conclusion, adrenal incidentalomas require a comprehensive evaluation that takes into account the possible clinical signs and comorbidities related to hormonal syndromes or malignancy; a complete hormone profile (taking into account the conditions that may lead to falsely positive and negative results); and an adequate radiographic study. Monitoring and/or treatment will be decided based on the results of the initial evaluation.

Neoplasia oncocítica adrenocortical de potencial maligno incierto: reporte de un caso / Adrenocortical oncocytic neoplasm with uncertain malignant potential: a case report

Resumen Objetivo Describir el caso clínico de una paciente con neoplasia oncocitica adrenocortical, tratado quirúrgicamente en una clínica de Lima, Perú. Caso clínico Paciente mujer de 26 años ingresa a emergencia por dolor abdominal inespecífico. Se evidencia tumoración de 15x14x12 cm dependiente de glándula suprarrenal izquierda por lo que se decide tratamiento quirúrgico. Al análisis patológico se evidencia neoplasia oncocítica de potencial maligno incierto. Discusión Las neoplasias oncocíticas adrenocorticales son entidades poco frecuentes, con escasos reportes de casos de esta enfermedad. Para clasificarlas, se usa la escala de Weiss modificada. Obtenemos una neoplasia oncocítica de potencial maligno incierto, cuyo tratamiento incluye la cirugía de resección de tumor y observación. Conclusión Considerar a las neoplasias oncocíticas dentro del diagnóstico diferencial de incidentalomas adrenales.

Objective To describe a case report of a oncocytic adrenocortical neoplasm, treated surgically in a clinic in Lima, Peru. Case report A 26-year-old woman is admitted to the emergency due to nonspecific abdominal pain. A tumor measuring 15x14x12 cm dependent on left adrenal gland is evidenced, so surgical treatment is decided. Pathological analysis evidences oncocytic neoplasia of uncertain malignant potential. Discussion Oncocytic adrenocortical neoplasms are rare entities, with few case reports of this disease. To classify them, the modified Weiss scale is used. We obtain an oncocytic neoplasm of uncertain malignant potential, whose treatment includes surgery for tumor resection and observation. Conclusion Consider oncocytic neoplasms within the differential diagnosis of adrenal incidentalomas.

Prevalencia de mielolipoma como diagnóstico incidental de masas adrenales en tomografías abdominales realizadas en el Hospital Universitario San Vicente Fundación de Medellín / Prevalence of adrenal myelolipoma during abdominal computed tomography at Hospital Universitario San Vicente Fundación

RESUMEN Objetivo: el mielolipoma es un tumor suprarrenal poco frecuente, benigno y no funcional. Representa el 2,6-3 % de los casos reportados de masas adrenales incidentales. Es más frecuente en adultos entre la quinta y séptima década de la vida. El objetivo del estudio es evaluar la prevalencia de mielolipoma como masa adrenal incidental en tomografías abdominales en 1 año, en un hospital universitario de cuarto nivel. Metodología: estudio retrospectivo. Se revisaron todas las tomografías de abdomen simples o contrastadas realizadas en el periodo 2014 al 2015 en el Hospital Universitario San Vicente Fundación de Medellín. De estas, se extrajeron las que reportaban masas adrenales incidentales con diagnóstico de mielolipoma. Se revisaron las historias clínicas de los pacientes con este hallazgo y sus características clínicas respectivas. Resultados: se revisaron 4.288 estudios tomográficos de abdomen correspondientes a 3.709 pacientes. Se encontraron 108 masas adrenales incidentales y los mielolipomas fueron el 1,85 % de estos. En los pacientes evaluados con TAC abdominal por diferentes razones, la posibilidad de identificar mielolipomas fue de 0,05 %. El promedio de edad de presentación fue 57 años. Ambos casos de mielolipomas fueron masas unilaterales, derechas, con un tamaño promedio de 31 mm y con un promedio de -84,5 unidades Hounsfield (UH). En ningún caso se realizó estudio hormonal o de manejo quirúrgico. Discusión: en este estudio se presenta la prevalencia y características clínicas del mielolipoma adrenal con presentación inicial como masas adrenales incidentales. Se encontró que el 1,85 % de las masas adrenales incidentales tienen como etiología el mielolipoma. En nuestro conocimiento no hay estudios en Colombia que evalúen la prevalencia de mielolipoma en TAC abdominales. En conclusión, el mielolipoma es un tumor adrenal infrecuente.

SUMMARY Objective: Adrenal myelolipoma is an infrequent benign tumor. Myelolipoma represents 2,6-3 % of all adrenal incidentalomas. This tumor is more frequent in aged individuals. Our aim was to evaluate the prevalence of myelolipoma in patients with adrenal incidentaloma detected by abdominal computed tomography (CT) during 1 year at a university tertiary care hospital. Methods: Retrospective study. All abdominal CT done at the Hospital Universitario San Vicente Fundación, Medellín, Colombia, with or without contrast, were reviewed from 2014 to 2015. Of all adrenal incidentalomas, those with diagnosis of adrenal mielolipoma were further evaluated. The clinical and laboratory data were extracted. Results: 4288 abdominal CT were reviewed in 3709 patients. 108 adrenal incidentalomas were found. Of those, two cases were adrenal myelolipomas. The frequency of mielolipoma presenting as adrenal incidentalomas was 1.85 % with a prevalence in abdominal CT of 0.05 %. The mean age at presentation was 57 years. Both cases were unilateral right masses; the mean size was 31 mm and a mean of -84.5 Hounsfield units. Hormonal studies or surgical management was no performed. Discussion: This study report the prevalence and clinical characteristics of adrenal myelolipoma presenting as adrenal incidentalomas. 1.85 % of adrenal incidentalomas are myelolipomas. To our knowledge, there are no studies in Colombia evaluating the prevalence of this disease in abdominal CT scans. In conclusion, myelolipoma is an infrequent adrenal tumor.

Mielolipoma suprarrenal: doce años de experiencia en su diagnóstico, tratamiento y seguimiento / Adrenal myelolipoma: twelve-years' experience in diagnosis, treatment and follow-up

Introducción: Los mielolipomas suprarrenales son tumores benignos e infrecuentes, formados por tejido adiposo y hematopoyético. Se consideran incidentalomas porque se diagnostican fortuitamente en estudios de imagen investigando síntomas abdominales o lumbares o en chequeos rutinarios. Son hormonalmente inactivos casi siempre. Se operan si presentan gran tamaño y usualmente se mantienen estables durante su evolución natural. Objetivos: Identificar las características de los mielolipomas suprarrenales y describir su evolución natural. Métodos: Se realizó un estudio descriptivo retrospectivo en 17 pacientes con mielolipomas suprarrenales diagnosticados por tomografía axial computarizada entre enero de 2006 y abril de 2018. Se estudiaron variables clínicas, hormonales y tomográficas al inicio en 17 pacientes y evolutivamente en 5 pacientes no operados. Se utilizaron medidas de resumen para las variables cualitativas (número y porcentajes) y para las cuantitativas (media y desviación estándar). Resultados: La edad promedio fue 52,9 años y la distribución por sexo: 13 mujeres y 4 hombres (razón 3,2:1). Se indicó tomografía axial computarizada en 11 pacientes por síntomas dolorosos. El tamaño promedio fue 5,6 cm. En 8 tumores el diámetro fue de 6 cm y más. En 9 pacientes se realizó adrenalectomía laparoscópica. Durante la evolución natural, que promedió 5 años y 1 mes, una paciente presentó crecimiento de sus dos masas bilaterales; la derecha se extirpó por sobrepasar los 6 cm. Conclusiones: Los mielolipomas suprarrenales fueron benignos, alcanzaron gran tamaño y provocaron síntomas dolorosos. Fueron hormonalmente inactivos en su mayoría. Evolutivamente, el crecimiento fue muy infrecuente y no hubo transformación maligna ni desarrollo de hiperfunción endocrina(AU)

Introduction: Suprarenal myelolipomas are infrequent benign tumors formed by adipose and hematopoietic tissue. They are considered to be incidentalomas because they are found and diagnosed accidentally in imaging studies intended for abdominal or lumbar problems, or in routine checkups. Suprarenal myelolipomas are almost always hormonally inactive. They are operated on when they are large, and they usually remain stable during their natural evolution. Objectives: Identify the characteristics of suprarenal myelolipomas and describe their natural evolution. Methods: A retrospective descriptive study was conducted of 17 patients with suprarenal myelolipomas diagnosed by computerized axial tomography from January 2006 to April 2018. Clinical, hormonal and tomographic variables were analyzed initially in 17 patients and evolutionarily in 5 non-operated patients. Summary measurements were used for qualitative variables (number and percentages) and for quantitative variables (mean and standard deviation). Results: Mean age was 52.9 years and sex distribution was 13 women and 4 men (ratio of 3.2:1). Computerized axial tomography was indicated for 11 patients with pain symptoms. Average size was 5.6 cm. In 8 tumors the diameter was 6 cm or more. Laparoscopic adrenalectomy was performed on 9 patients. During natural evolution, which averaged 5 years and 1 month, one female patient experienced growth of her two bilateral masses, and the one on the right side was removed for it exceeded 6 cm. Conclusions: The study suprarenal myelolipomas were large, benign and caused pain symptoms. Most were hormonally inactive. In evolutionary terms, growth was very infrequent and there was no malignant transformation or development of endocrine hyperfunction(AU)

Mielolipoma suprarrenal: doce años de experiencia en su diagnóstico, tratamiento y seguimiento / Adrenal myelolipoma: twelve-years' experience in diagnosis, treatment and follow-up

Introducción: Los mielolipomas suprarrenales son tumores benignos e infrecuentes, formados por tejido adiposo y hematopoyético. Se consideran incidentalomas porque se diagnostican fortuitamente en estudios de imagen investigando síntomas abdominales o lumbares o en chequeos rutinarios. Son hormonalmente inactivos casi siempre. Se operan si presentan gran tamaño y usualmente se mantienen estables durante su evolución natural. Objetivos: Identificar las características de los mielolipomas suprarrenales y describir su evolución natural. Métodos: Se realizó un estudio descriptivo retrospectivo en 17 pacientes con mielolipomas suprarrenales diagnosticados por tomografía axial computarizada entre enero de 2006 y abril de 2018. Se estudiaron variables clínicas, hormonales y tomográficas al inicio en 17 pacientes y evolutivamente en 5 pacientes no operados. Se utilizaron medidas de resumen para las variables cualitativas (número y porcentajes) y para las cuantitativas (media y desviación estándar). Resultados: La edad promedio fue 52,9 años y la distribución por sexo: 13 mujeres y 4 hombres (razón 3,2:1). Se indicó tomografía axial computarizada en 11 pacientes por síntomas dolorosos. El tamaño promedio fue 5,6 cm. En 8 tumores el diámetro fue de 6 cm y más. En 9 pacientes se realizó adrenalectomía laparoscópica. Durante la evolución natural, que promedió 5 años y 1 mes, una paciente presentó crecimiento de sus dos masas bilaterales; la derecha se extirpó por sobrepasar los 6 cm. Conclusiones: Los mielolipomas suprarrenales fueron benignos, alcanzaron gran tamaño y provocaron síntomas dolorosos. Fueron hormonalmente inactivos en su mayoría. Evolutivamente, el crecimiento fue muy infrecuente y no hubo transformación maligna ni desarrollo de hiperfunción endocrina(AU)

Introduction: Suprarenal myelolipomas are infrequent benign tumors formed by adipose and hematopoietic tissue. They are considered to be incidentalomas because they are found and diagnosed accidentally in imaging studies intended for abdominal or lumbar problems, or in routine checkups. Suprarenal myelolipomas are almost always hormonally inactive. They are operated on when they are large, and they usually remain stable during their natural evolution. Objectives: Identify the characteristics of suprarenal myelolipomas and describe their natural evolution. Methods: A retrospective descriptive study was conducted of 17 patients with suprarenal myelolipomas diagnosed by computerized axial tomography from January 2006 to April 2018. Clinical, hormonal and tomographic variables were analyzed initially in 17 patients and evolutionarily in 5 non-operated patients. Summary measurements were used for qualitative variables (number and percentages) and for quantitative variables (mean and standard deviation). Results: Mean age was 52.9 years and sex distribution was 13 women and 4 men (ratio of 3.2:1). Computerized axial tomography was indicated for 11 patients with pain symptoms. Average size was 5.6 cm. In 8 tumors the diameter was 6 cm or more. Laparoscopic adrenalectomy was performed on 9 patients. During natural evolution, which averaged 5 years and 1 month, one female patient experienced growth of her two bilateral masses, and the one on the right side was removed for it exceeded 6 cm. Conclusions: The study suprarenal myelolipomas were large, benign and caused pain symptoms. Most were hormonally inactive. In evolutionary terms, growth was very infrequent and there was no malignant transformation or development of endocrine hyperfunction(AU)

Incidentaloma adrenal Estado del arte / Adrenal incidentaloma State of the art

Propósito: el uso creciente de las imágenes diagnósticas ha permitido descubrir lesiones adrenales en pacientes sin sospecha previa de una enfermedad originada en las mismas, hallazgo conocido como incidentaloma suprarrenal. Nuestro propósito es presentar una revisión del tema para la actualización del personal médico que se encuentre ante un paciente con esta entidad. Fuente de los datos: se realizó una búsqueda con el término MESH "adrenal incidentaloma" en español, portugués e inglés en la base de datos Medline desde el año 2000. Se obtuvieron 352 referencias. Se realizó una búsqueda regional de la literatura con el término "incidentaloma" en las bases de datos Scielo, Lilacs y Bireme. Se excluyeron las referencias a incidentalomas hipofisiarios, hepáticos y tiroideos. En Lilacs se encontraron 42 referencias y se excluyó un artículo por tratarse de incidentalomas hepáticos. En Scielo se encontraron 37 referencias y se excluyeron cinco por tratarse de incidentalomas tiroideos, hipofisiarios y una referencia a carcinoma renal. La mayoría de referencias en nuestra región son de reportes de casos y revisiones con pocos estudios originales. Selección de los estudios: se excluyeron aquellos estudios que se referían a incidentalomas en otros órganos. Resultados de la síntesis de datos: los incidentalomas adrenales pueden ser lesiones benignas como los mielolipomas, infecciones por hongos y micobacterias o lesiones infrecuentes como los schwanomas. También se pueden encontrar adenomas, los cuales pueden ser productores de hormonas o no productores. También se pueden encontrar lesiones malignas, como carcinomas adrenales y metástasis. Ante la presencia de un incidentaloma suprarrenal con tamaño mayor de 4 cm, irregularidad en los bordes, alta atenuación de la lesión (en unidades Hounsfield) y un bajo porcentaje de lavado en el estudio contrastado se debe sospechar carcinoma adrenal, en cuyo caso el manejo es quirúrgico, una vez se haya descartado feocromocitoma. Ahora bien, si la lesión no sugiere malignidad, el clínico debe preguntarse si el incidentaloma es productor o no de hormonas. En este sentido se debe evaluar la posibilidad de síndrome de Cushing (mediante una prueba de supresión con dosis bajas de dexametasona), feocromocitoma (con medición de metanefrinas en orina o en plasma) y si es hipertenso descartar hiperaldosteronismo primario midiendo la relación aldosterona/renina. Acorde a estos resultados se definirá la conducta quirúrgica o el seguimiento clínico e imagenológico. Conclusiones: el incidentaloma adrenal se ha convertido en una enfermedad frecuente en la práctica clínica cotidiana del internista. Aunque la mayoría de estas lesiones no son malignas y corresponden a adenomas no productores, siempre debe considerarse basado en las características clínicas y paraclínicas la posibilidad de una enfermedad endocrina. Se requieren estudios para conocer la epidemiología y la etiología de esta enfermedad en nuestro medio. (Acta Med Colomb 2015; 40: 318-325).

Purpose: increased use of diagnostic imaging has led to the discovery of adrenal masses in patients without prior suspicion of a disease originated in adrenals, known as adrenal incidentaloma finding. Our purpose is to present a review of the topic to update the medical staff faced with a patient with this condition. Data Source: a search with the MeSH term "adrenal incidentaloma" was conducted in Spanish, Portuguese and English in the Medline database since 2000. 352 references were obtained. Regional literature search with the term "incidentaloma" on Scielo, Lilacs and Bireme databases was performed. References to pituitary, liver and thyroid incidentalomas were excluded. 42 references were found in Lilacs and 1 article was excluded because they were liver incidentalomas. 37 references were found in Scielo and 5 were excluded, being treated thyroid, pituitary incidentalomas and a reference to renal carcinoma. Most references in our region are case reports and reviews with few original studies. Study Selection: studies that referred to incidentalomas in other organs were excluded. Results of data synthesis: adrenal incidentalomas can be benign like myelolipomas, fungal and mycobacterial infections or infrequent lesions as schwannomas. Adenomas which can be hormone producing and non-producing hormones can also be found. Also malignant lesions such as adrenal carcinomas and metastases can be found. In the presence of an adrenal incidentaloma larger than 4 cm, irregular edges, high attenuation of the lesion (in Hounsfield units) and a low percentage of washing in the contrast study, adrenal carcinoma should be suspected, in which case management is surgical once pheochromocytoma has been excluded. However, if the injury does not suggest malignancy, the clinician should wonder whether incidentaloma is or is not hormone- producing. In this regard we must assess the possibility of Cushing's syndrome (via a suppression test with low doses of dexamethasone), pheochromocytoma (with measurement of metanephrines in urine or plasma) and if the patient is hypertensive, discard primary aldosteronism by measuring the aldosterone / renin ratio. According to these results the surgical treatment or clinical and imaging follow-up will be defined. Conclusions: The adrenal incidentaloma has become a common disease in daily clinical practice of the internist. Although most of these lesions are not malignant and correspond to non-producing adenomas, the possibility of an endocrine disease should always be considered based on clinical and paraclinical features. Studies to acknowledge the epidemiology and etiology of this disease in our environment are required. (Acta Med Colomb 2015; 40: 318-325).

Linfoma adrenal primário apresentando-se como incidentaloma adrenal: relatode caso e revisão da literatura / Primary adrenal lymphoma presenting as an adrenal incidentaloma: case report and review of the literature

Introdução: O linfoma primário de glândulas adrenaisé um evento raro. Devido à agressividade dessetumor, a forma mais comum de apresentação é a insuficiênciaadrenal primária, sendo muito infrequente aapresentação na forma de um incidentaloma adrenal.Incidentalomas adrenais correspondem a tumores sóidentificados após a realização de exames de imagempara esclarecimento de alguma anormalidade semorigem, a princípio, nas glândulas adrenais. Objetivo:Descrever o caso de um paciente de 61 anos portadorde linfoma difuso de grandes células B com acometimentoprimário bilateral de glândulas adrenais, este seapresentando na forma de um incidentaloma adrenal.Material e Métodos: Relato do caso, revisão de examese discussão baseada na literatura. Os artigos selecionadospara a discussão foram buscados no PubMed edatam dos últimos 12 anos. Resultados: Paciente masculino,61 anos, previamente hígido, apresentou-se emconsulta ambulatorial com queixa de dor abdominaldifusa, perda de peso e astenia associados. Exames deimagem evidenciaram aumento volumétrico de ambasas adrenais. Não havia sintomas ou sinais clínicos sugestivosde hipofunção ou hiperfunção adrenal e osexames laboratoriais iniciais descartaram a presençade insuficiência adrenal, feocromocitoma, hiperaldosteronismoprimário e síndrome de Cushing. O pacienteevoluiu com queda do estado geral, vômitos e confusãomental, sendo diagnosticada crise adrenal, confirmadaapós laboratorialmente. O estudo histológico evidenciouneoplasia maligna indiferenciada, sendo definidaa presença de linfoma difuso de grandes células B naavaliação imunohistoquímica. Conclusões: Apesar deser uma condição rara, o linfoma primário de glândulasadrenais deve ser lembrado como um possível diagnósticono contexto de incidentaloma adrenal.

Introduction: Primary adrenal lymphoma is a rare event,Due to the agressiveness of this tumor, the most commonform of presentation is primary adrenal insufficiency ?presentation as an adrenal incidentaloma is very unusual.Adrenal incidentalomas are turmors only indentified afterradiologic evaluation in order to clarify some abnormalitythat is not originated, at the first instance, in adrenalglands. Objective: To describe a case of a 61-year-old patientwith lymphoma diffuse large B-cell involving adrenalglands bilaterally and presenting as an adrenal incidentaloma.Methods: Case report, review of tests and discussionbased on the literature. The articles choosen to thediscussion were searched in PubMed and date from thelast twelve years. Results: A 61 year-old male patient, helathypreviously, presented in outpatient service with diffuseabdominal pain, wheight loss and astenia associated.Imaging studies revealed an increase in volume of bothadrenal glands. There were no clinical signs or symptonssuggestive of adrenal hipofunction or hyperfunction andinitial laboratorial tests ruled out the presence of adrenalinsufficiency, pheocromocytoma, primary aldosteronismand Cushing?s syndrome. The patient developed then adecline in general status, emesis and mental confusionand an adrenal crisis was confirmed. Histologic study ofthe adrenal mass demonstrated an undifferenciated malignantneoplasm and the immunohistochemical evaluationshowed a large B-cell diffuse lymphoma. Conclusions:Despite being a rare condition, primmary adrenal lymphomashould be considered as a possible diagnosis in theclinical context of an adrenal incidentaloma.

Mielolipomas adrenales bilaterales como hallazgo incidental / Bilateral adrenal myelolipomas as an incidental finding

Los mielolipomas son tumores benignos raros, no funcionantes, compuestos por tejido adiposo maduro y precursores hematopoyéticos que involucran principalmente a las glándulas adrenales aunque pueden presentarse en localizaciones extraadrenales. Las características clínicas son muy variables, con descripciones de mielolipomas gigantes, bilaterales, asociados a síndromes de hipersecreción hormonal o a sangrado retroperitoneal. Con el incremento de la utilización de técnicas de imagen abdominales en la práctica clínica habitual, se ha observado un aumento de detección de incidentalomas adrenales, entre los cuales pueden encontrarse en raras ocasiones mielolipomas. Se describe el caso de una presentación inusual de mielolipomas adrenales bilaterales asintomáticos como hallazgo incidental en radiografías convencionales de abdomen, junto con un revisión de la literatura referida a estos tumores.

Myelolipomas are rare, non-functioning, benign tumors composed of mature fat tissue and myeloid hematopoietic precursors that involve mainly the adrenal glands and rarely other extra-adrenal tissues. The clinical features are heterogeneous and there are reports of giant and bilateral myelolipomas. Few tumors have been associated with endocrine dysfunction or retroperitoneal bleeding. With the development and wide-spread use of modern imaging techniques, this adrenal entity is sometimes found incidentally during radiology procedures for other reasons. We describe an unusual clinical presentation of asymptomatic bilateral myelolipomas incidentally discovered in abdominal X-Rays.

Ovarian hyperthecosis in the context of an adrenal incidentaloma in a postmenopausal woman / Coexistência de incidentaloma adrenal e hipertecose de ovário em mulher menopausada

Adrenal incidentaloma is not infrequent and can be found in hirsute women. We report a case of a 54-year-old woman with amenorrhea and hirsutism of abrupt onset and mild signs of virilization that had an adrenal incidentaloma coexisting with ovarian hyperthecosis. Basal total and free testosterone were 191 ng/dL and 179 pmol/L. Pelvic ultrasonography disclosed a right ovary with 10.3 cc and a left ovary with 9.8 cc without nodules or cysts, and computerized tomography of the abdomen disclosed a normal right adrenal gland. On the left adrenal gland a solid nodule with 0.8 cm was seen. After GnRHa administration, total testosterone was 23 ng/dL and free testosterone was 17 pmol/L. In view of a suppression of testosterone by GnRHa, the patient was submitted to a hystero-oophorectomy by laparoscopy. Symmetrically enlarged ovaries were seen. No tumor was apparent. Histology showed hyperthecosis, with foci of luteinized stromal cells. Only atretic follicles were detected. No hilar cell hyperplasia was seen. In conclusion, the presence of an adrenal mass in a hirsute woman can lead to a wrong diagnosis. In this case the suppression GnRHa test was fundamental to determine the origin of hyperandrogenemia.

Os incidentalomas adrenais não são infreqüentes e podem ser encontrados em pacientes com hirsutismo. Nesse relato, apresentamos o caso de coexistência de um incidentaloma adrenal com hipertecose de ovário, em uma mulher com 54 anos de idade com amenorréia e hirsutismo de início abrupto e sinais leves de virilização. As testosteronas total e livre basal foram de 191 ng/dL e 179 pmol/L, respectivamente. O ultra-som pélvico demonstrou o ovário direito com 10,3 cc e ovário esquerdo com 9,8 cc, sem nódulos ou cistos e a tomografia computadorizada de abdome demonstrou adrenal direita adrenal e nódulo sólido de 0,8 cm na adrenal esquerda. Após a administração de análogo de GnRH, as testosteronas total e livre foram de 23 ng/dL e 17 pmol/L, respectivamente. Considerando a supressão da concentração de testosterona pelo análogo de GnRH, a paciente foi submetida a histeroooforectomia por via laparoscópica. O diagnóstico histológico foi de hipertecose, com focos de células estromais luteinizadas. Somente folículos atréticos foram visualizados. Não se detectou hiperplasia de células hilares. Em conclusão, a presença de massa adrenal em uma paciente com hirsutismo pode levar ao diagnóstico errado. Neste caso, o teste de supressão com análogo de GnRH foi fundamental para se determinar a origem da hiperandrogenemia.

Estudio de 34 pacientes con incidentaloma suprarrenal / A study of 34 cases of adrenal incidentaloma

El incidentaloma suprarrenal, un tumor de dicha glándula descubierto por razones independientes de la sospecha de enfermedad adrenal, constituye un problema clínico frecuente. Aunque en la mayoría de los casos son benignos y no hiperfuncionantes, es importante identificar oportunamente la minoría de lesiones malignas o hiperfuncionantes de resolución quirúrgica. Si bien han sido diseñadas distintas estrategias de diagnóstico hay controversia alrededor de una serie de cuestiones. En el presente trabajo retrospectivo once (32%) de nuestros 34 pacientes presentaban masas adrenales hiperfuncionantes manifestadas por síndrome de Cushing subclínico en cuatro, feocromocitoma en tres, probable hiperaldosteronismo primario en dos y por hiperplasia adrenal congénita de origen tardío y carcinoma funcionante en los dos restantes. Las características de las imágenes por TAC y/o RM permitieron identificar los adenomas a la vez que decidir la cirugía tanto en dos pacientes con feocromocitomas bioquímicamente no funcionantes como en una paciente con un carcinoma adrenocortical primitivo, este diagnóstico también sugerido por un patrón mixto de hipersecreción de andrógenos y cortisol. En una paciente con síndrome de Cushing subclínico, hipertensa y diabética, ambas comorbilidades fueron resueltas por la cirugía. Los tumores no funcionantes fueron en su mayoría adenomas (87%), hallándose además histoplasmosis, pseudoquiste, hiperplasia suprarrenal idiopática y mielolipoma. Seis de los ocho pacientes operados tenían enfermedad maligna y/o hiperfuncionante. La condición asociada a los incidentalomas suprarrenales significó un amplio espectro de riesgo para los pacientes y reafirma la necesidad de una minuciosa evaluación clínica, bioquímica y de las imágenes a fin de adoptar conductas adecuadas.

Adrenal incidentaloma, a tumor discovered unexpectedly during imaging performed for non-adrenal related causes, has become a frequent clinical concern. Although in most cases they are benign and hormonally nonfunctioning, it is important to appropriately identify those few cases of malignant or hyperfunctioning lesions of surgical resolution. Although several proposals for an optimal diagnostic strategy have been designed, controversy over a series of questions still persists. In the present retrospective study we analyzed 34 patients with adrenal incidentaloma. Of these, 32% of the patients displayed hyperfunctioning pathologies that included subclinical Cushing's syndrome in four patients, probable primary hyperaldosteronism in two, late onset congenital adrenal hyperplasia in one, adrenocortical carcinoma in one and pheochromocytoma in three. CT and/or MRI permitted the identification of adenomas and were crucial to decide on surgery in two patients with nonfunctioning pheochromocytomas and in a patient carrying a primitive adrenocortical carcinoma, a diagnosis also suggested by a mixed pattern of hypersecretion of androgens and cortisol. In a diabetic and hypertensive patient with subclinical Cushing's syndrome both comorbidities were solved by surgery. Nonfunctioning tumors were mainly adenomas (87%) with individual cases of histoplasmosis, pseudocyst, idiopathic adrenal hyperplasia and mielolipoma. Six of the eight operated patients presented malignant and/or hyperfunctioning tumors. The pathology associated with incidentalomas represents a broad spectrum of risk for patients and reaffirms the necessity for a meticulous clinical, biochemical, and imaging evaluation in order to make appropriate decisions.

Crisis hipertensiva por punción biopsia de feocromocitoma adrenal: a propósito de un caso / Hypertensive crisis due to puncture biopsy of adrenal pheochromocytoma: a case report

Se presenta el caso de un paciente adulto masculino que sufrió una crisis hipertensiva por la punción de un incidentaloma suprarrenal. Era portador de una neoplasia endócrina múltiple tipo 2A (NEM 2A). Se describe la utilidad del diagnóstico para descartar un feocromocitoma en el momento de decidir una práctica invasiva como la biopsia. Se analiza el dosaje de metabolitos de las catecolaminas [normetanefrina (NMN), metanefrina (MN) y ácido vainillín mandélico (AVM)] como método diagnóstico de certeza. Se plantea la ventaja del tratamiento preventivo para evitar la crisis hipertensiva descripta en este paciente.

The case of an adult male patient who suffered a hypertensive crisis following an adrenal incidentaloma biopsy is presented in this paper. This patient had a type IIA multiple endocrine neoplasm (MEN IIA). Before using an invasive procedure for the incidentally discovered adrenal mass, it is necessary to biochemically rule out a possible pheochromocytoma. The use of alpha-blockers could be indicated in order to avoid complications. Catecholamine metabolite level ­normetanephrine (NMN), metanephrine (MN), and vanillylmandelic acid (VMA)- is analyzed as an accurate diagnostic tool. The advantage of preventive treatment in order to avoid the hypertensive crisis described in this patient is also outlined in this paper.