RESUMO

El carcinoma suprarrenal es una enfermedad muy poco frecuente de presentación clínica poco específica, donde los síntomas por la propia neoplasia son los predominantes. El objetivo de esta publicación es presentar un caso clínico jerarquizando los estudios de imagen para el diagnóstico y la planificación de la táctica para la cirugía.

Adrenal carcinoma is a very rare disease with nonspecific clinical presentation, where symptoms from the neoplasia itself are predominant. The aim of this publication is to present an uncommon clinical case, emphasizing the role of imaging studies in diagnosis and surgical strategy planning.

O carcinoma adrenal é uma doença muito rara, com apresentação clínica inespecífica onde predominam os sintomas decorrentes da própria neoplasia. O objetivo desta publicação é apresentar um caso clínico infrequente priorizando exames de imagem para diagnóstico e planejamento de táticas cirúrgicas.

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Resumen Los carcinomas adrenocorticales son tumores infre cuentes, habitualmente hiperfuncionantes y con una supervivencia global pobre. La edad frecuente de pre sentación se describe en adultos entre 40 a 60 años, con predominio en sexo femenino. Se presentan dos casos inusuales de carcinoma adrenal diagnosticados en mujeres en edad fértil. El primero de ellos se descubrió en el segundo trimestre de gestación, con un cuadro de hipercortisolismo y lesión adrenal localizada, que resolvió con resección completa hacia la semana 20. En el segundo, la paciente debutó con manifestaciones clínicas de virilización rápidamente progresiva, sien do el hiperandrogenismo puro el patrón bioquímico hallado. En ambos casos, a pesar de haberse realizado la resección completa, el Ki67 elevado como principal factor pronóstico condujo a categorizarlas como de "alto riesgo de recurrencia". Asimismo, se ha asociado a la gestación y al patrón secretor de glucocorticoides como factores adicionales de mayor riesgo de recurrencia. Este es particularmente elevado dentro de los dos primeros años posteriores al diagnóstico. Existe aún controversia sobre el uso de mitotane adyuvante en estos pacientes, y su inicio está recomendado hasta los tres meses del postquirúrgico. Sin embargo, la evidencia disponible no permite suponer la falta de eficacia si se utiliza fuera de ese período. Los limitantes, como fueron el curso de la gestación y el puerperio inmediato, así como la difi cultad para el acceso a la medicación en nuestro medio, impidieron el inicio precoz del tratamiento adyuvante en ambos casos, aunque surge la inquietud de si aún sería oportuna su instauración.

Abstract Adrenocortical carcinomas are rare tumors, usually hyperfunctioning, with poor overall survival. Frequent age of presentation is described in adults between 40 and 60 years of age, predominantly female. Two unusual cases of adrenal carcinoma diagnosed in young women are presented. The first one was discovered in the sec ond trimester of gestation, with signs and symptoms of hypercortisolism and localized adrenal lesion, which was resolved with complete resection by week 20 of pregnancy. In the second case, the patient begined with clinical manifestations of rapidly progressive virilization, the biochemical pattern being pure hyperandrogenism. In both cases, despite complete resection, the high Ki67 as the main prognostic factor leaded to categorization as "high risk of recurrence". In addition, pregnancy and glucocorticoid secretory pattern have been associated as additional risk factors of recurrence. This is particularly high within the first two years after diagnosis. There is controversy about the use of adjuvant mitotane in these patients, and the general recommendation is to be started no longer than 3 months after surgery. However, the available evidence does not suggest that its use is ineffective beyond that period. Limitations, such as the course of pregnancy and the immediate puerperium, as well as the difficulty of accessing this medication in our environment, prevented the early initiation of adjuvant treatment with mitotane in both cases, although there is still concern whether its administration would still be appropriate.

RESUMO

Adrenocortical carcinomas are rare tumors, usually hyperfunctioning, with poor overall survival. Frequent age of presentation is described in adults between 40 and 60 years of age, predominantly female. Two unusual cases of adrenal carcinoma diagnosed in young women are presented. The first one was discovered in the second trimester of gestation, with signs and symptoms of hypercortisolism and localized adrenal lesion, which was resolved with complete resection by week 20 of pregnancy. In the second case, the patient begined with clinical manifestations of rapidly progressive virilization, the biochemical pattern being pure hyperandrogenism. In both cases, despite complete resection, the high Ki67 as the main prognostic factor leaded to categorization as "high risk of recurrence". In addition, pregnancy and glucocorticoid secretory pattern have been associated as additional risk factors of recurrence. This is particularly high within the first two years after diagnosis. There is controversy about the use of adjuvant mitotane in these patients, and the general recommendation is to be started no longer than 3 months after surgery. However, the available evidence does not suggest that its use is ineffective beyond that period. Limitations, such as the course of pregnancy and the immediate puerperium, as well as the difficulty of accessing this medication in our environment, prevented the early initiation of adjuvant treatment with mitotane in both cases, although there is still concern whether its administration would still be appropriate.

Los carcinomas adrenocorticales son tumores infrecuentes, habitualmente hiperfuncionantes y con una supervivencia global pobre. La edad frecuente de presentación se describe en adultos entre 40 a 60 años, con predominio en sexo femenino. Se presentan dos casos inusuales de carcinoma adrenal diagnosticados en mujeres en edad fértil. El primero de ellos se descubrió en el segundo trimestre de gestación, con un cuadro de hipercortisolismo y lesión adrenal localizada, que resolvió con resección completa hacia la semana 20. En el segundo, la paciente debutó con manifestaciones clínicas de virilización rápidamente progresiva, siendo el hiperandrogenismo puro el patrón bioquímico hallado. En ambos casos, a pesar de haberse realizado la resección completa, el Ki67 elevado como principal factor pronóstico condujo a categorizarlas como de "alto riesgo de recurrencia". Asimismo, se ha asociado a la gestación y al patrón secretor de glucocorticoides como factores adicionales de mayor riesgo de recurrencia. Este es particularmente elevado dentro de los dos primeros años posteriores al diagnóstico. Existe aún controversia sobre el uso de mitotane adyuvante en estos pacientes, y su inicio está recomendado hasta los tres meses del postquirúrgico. Sin embargo, la evidencia disponible no permite suponer la falta de eficacia si se utiliza fuera de ese período. Los limitantes, como fueron el curso de la gestación y el puerperio inmediato, así como la dificultad para el acceso a la medicación en nuestro medio, impidieron el inicio precoz del tratamiento adyuvante en ambos casos, aunque surge la inquietud de si aún sería oportuna su instauración.

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

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

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RESUMEN Introducción: Las glándulas suprarrenales se dividen en corteza y médula. La corteza secreta tres clases de hormonas diferentes: los glucocorticoides, mineralocorticoides y andrógenos; la médula produce las catecolaminas. Todas ellas intervienen en múltiples funciones corporales. Objetivo: Mostrar diferentes formas de presentación de algunas de las enfermedades de la corteza adrenal. Presentación de los casos: Paciente 1: escolar de 7 años, antecedentes de hipotiroidismo controlado, presenta decaimiento y escaso crecimiento pondoestatural. Cortisol en ayunas 86 nmol/L y ACTH 154 pg/ml. Se diagnostica insuficiencia adrenal primaria. Reingresa a los 9 años, por mantener escaso crecimiento. Edad ósea: 2 años y 8 meses. Test de clonidina: alterado, confirma déficit de hormona del crecimiento. Paciente 2: escolar de 8 años, antecedentes de hiperplasia adrenal congénita por déficit de 21 hidroxilasa, forma virilizante simple. Presenta aceleración del desarrollo genital. Se incumplió tratamiento con hidrocortisona, durante 5 años. Se comprobó elevada la 17 hidroxiprogesterona 189 ng/dl. Se diagnosticó pubertad precoz periférica y virilización de los genitales. Paciente 3: lactante de 5 meses con obesidad de 3 meses de evolución. Tomografía axial computarizada de abdomen contrastado confirma lesión tumoración de 5 cm en proyección de la suprarrenal derecha. Cortisol en ayunas 892 nmol/L, cortisol 11 pm 920 nmol/L. Se realizó cirugía y se diagnosticó síndrome de Cushing por carcinoma adrenal productor de cortisol. Conclusiones: En la práctica clínica diaria podemos encontrar pacientes con síntomas y signos tan comunes como el decaimiento, vómitos o el aumento brusco de peso y ellos ser secundario a enfermedades de la glándula suprarrenal.

ABSTRACT Introduction: The adrenal glands are divided into cortex and marrow. The cortex secretes three different classes of hormones: glucocorticoids, mineralocorticoids and androgens; the marrow produces catecholamines. All of them are involved in multiple corporal functions. Objective: Show different forms of presentation of some of the diseases of the adrenal cortex. Presentation of cases: Patient 1: 7-year-old school boy, history of controlled hypothyroidism, and presenting decay and poor pondostatural growth. Cortisol level in fasting 86 nmol/L and ACTH 154 pg/ml. Primary adrenal insufficiency is diagnosed. Re-admitted at age 9 for maintaining little growth. Bone age: 2 years and 8 months. Clonidine test: altered, it confirms growth hormone deficiency. Patient 2: 8-year-old schoolboy, history of congenital adrenal hyperplasia due to 21 hydroxylase deficiency, simple virilizing form. He presents acceleration of genital development. Hydrocortisone treatment was missed for 5 years. 17 hydroxyprogesterone was found to be elevated to 189 ng/dl. Peripheral precocious puberty and virilization of the genitals were diagnosed. Patient 3: 5-month-old infant with obesity of 3 months of evolution. Contrasted computed axial tomography of the abdomen confirms tumor lesion of 5 cm in projection of the right adrenal. Cortisol level in fasting 892 nmol/L, cortisol 11 pm 920 nmol/L. Surgery was performed and Cushing's syndrome was diagnosed due to cortisol-producing adrenal carcinoma. Conclusions: In daily clinical practice we can find patients with symptoms and signs as common as decay, vomiting or sudden weight gain and they are secondary to diseases of the adrenal gland.

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ABSTRACT: In cattle, abdominal tumors leading to partial or total intestinal obstruction are rare. Here, it is described the clinico-pathological findings of two cows with intestinal obstruction by neoplasms. A crossbred cow presented apathy, weight loss, constipation, enophthalmos, ruminal and intestinal hypomotility, and abdominal distension in the right ventral quadrant. Pathological examination revealed bilateral ovarian teratoma. The other case was a cow that in the past 2 years had several episodes of squamous cell carcinoma in the third eyelid and vulva. During the 6 days prior to investigation, clinical signs of constipation and tenesmus were noted. Nodular and friable masses were located in the vulva, right third eyelid and cranially to the right kidney that were identified as poorly differentiated metastatic carcinoma of the adrenal cortex. To our knowledge, these are probably the first reports of ovarian teratoma and adrenal carcinoma causing clinical signs of intestinal obstruction in cattle.

RESUMO: Em bovinos, tumores abdominais que levam à obstrução intestinal parcial ou total são raros. Neste relato, descrevemos os achados clínico-patológicos de duas vacas com obstrução intestinal por neoplasias. Uma vaca mestiça apresentava apatia, perda de peso, constipação, enoftalmia, hipomotilidade ruminal e intestinal e distensão abdominal no quadrante ventral direito. O exame patológico revelou teratoma ovariano bilateral. O outro caso foi uma vaca que, nos últimos 2 anos, teve episódios de carcinoma de células escamosas na terceira pálpebra e vulva. Durante 6 dias antes da investigação, foram observados constipação e tenesmo. Massas nodulares e friáveis foram localizados na vulva, terceira pálpebra direita e cranial ao rim direito, que foram identificadas como carcinoma metastático pouco diferenciado do córtex adrenal. Ao nosso conhecimento, estes são os primeiros relatos de teratoma ovariano e carcinoma adrenal causando sinais clínicos de obstrução intestinal em bovinos.

RESUMO

In cattle, abdominal tumors leading to partial or total intestinal obstruction are rare. Here, it is described the clinico-pathological findings of two cows with intestinal obstruction by neoplasms. A crossbred cow presented apathy, weight loss, constipation, enophthalmos, ruminal and intestinal hypomotility, and abdominal distension in the right ventral quadrant. Pathological examination revealed bilateral ovarian teratoma. The other case was a cow that in the past 2 years had several episodes of squamous cell carcinoma in the third eyelid and vulva. During the 6 days prior to investigation, clinical signs of constipation and tenesmus were noted. Nodular and friable masses were located in the vulva, right third eyelid and cranially to the right kidney that were identified as poorly differentiated metastatic carcinoma of the adrenal cortex. To our knowledge, these are probably the first reports of ovarian teratoma and adrenal carcinoma causing clinical signs of intestinal obstruction in cattle.(AU)

Em bovinos, tumores abdominais que levam à obstrução intestinal parcial ou total são raros. Neste relato, descrevemos os achados clínico-patológicos de duas vacas com obstrução intestinal por neoplasias. Uma vaca mestiça apresentava apatia, perda de peso, constipação, enoftalmia, hipomotilidade ruminal e intestinal e distensão abdominal no quadrante ventral direito. O exame patológico revelou teratoma ovariano bilateral. O outro caso foi uma vaca que, nos últimos 2 anos, teve episódios de carcinoma de células escamosas na terceira pálpebra e vulva. Durante 6 dias antes da investigação, foram observados constipação e tenesmo. Massas nodulares e friáveis foram localizados na vulva, terceira pálpebra direita e cranial ao rim direito, que foram identificadas como carcinoma metastático pouco diferenciado do córtex adrenal. Ao nosso conhecimento, estes são os primeiros relatos de teratoma ovariano e carcinoma adrenal causando sinais clínicos de obstrução intestinal em bovinos.(AU)

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Introducción: el carcinoma suprarrenal primario es un tumor poco frecuente, altamente agresivo, de crecimiento rápido, con mayor incidencia entre los 40 y 60 años de edad. Los carcinomas funcionantes representan hasta un 79 por ciento de los tumores corticales, más frecuentes en el sexo femenino, y de estos el 50 por ciento se manifiestan clínicamente como un síndrome de Cushing. La extensión del tumor a estructuras vecinas es común y empeora el pronóstico. La supervivencia media es de 2 años desde el diagnóstico, en particular, cuando existen metástasis en hígado y pulmón. Objetivo: describir las características clínicas, los procederes diagnósticos y terapéuticos de una paciente con carcinoma adrenal de rápida evolución. Presentación del caso: paciente LRS, femenina, blanca, de 49 años de edad, con antecedentes de hipertensión arterial y diabetes mellitus tipo 2, que asiste a consulta por descontrol metabólico y de la hipertensión arterial. Al examen físico, se constatan signos sugestivos de hipercortisolismo, sintomatología que a los 2 meses se acentuó notablemente. Se realizaron estudios basales, dinámicos, imagenológicos y anatomopatológicos, que corroboraron el diagnóstico presuntivo. Se realiza adrenalectomía izquierda con adenectomía regional, y se confirma por anatomía patológica el diagnóstico de carcinoma suprarrenal izquierdo. A los pocos meses de la intervención la paciente fallece con metástasis óseas en columna vertebral. Conclusiones: el reconocimiento temprano de los síntomas y signos de hiperfunción adrenal es muy importante para el diagnóstico y tratamiento oportuno del carcinoma adrenal(AU)

Introduction: primary suprarrenal carcinoma is a highly aggressive rare carcinoma of rapid growth, with greater incidence in 40-60 years age group. The functioning carcinomas represent up to 79 percent of cortical tumors that are more frequent in females, and 50 percent of them clinically manifests as Cushing syndrome. The extension of the tumor to neighboring structures is common and worsens the prognosis. Mean survival rate is 2 years from the time of diagnosis, particularly when there are liver and lung metastases. Objective: to describe the clinical characteristics, the diagnostic and therapeutic procedures in a female patient with rapidly evolving adrenal carcinoma. Case presentation: a patient LRS, female, Caucasian, 49 years-old, with a history of blood hypertension and type 2 diabetes mellitus, who went to the hospital because of lack of metabolic control and blood hypertension. On physical examination, there were observed some signs suggestive of hypercortisolism, symptomatology that became notably acute two months later. Basal, dynamic, imaging and anatomopathological studies were made to corroborate the presumptive diagnosis. Left adrenalectomy with regional adenectomy was performed, and the diagnosis of left suprarenal carcinoma was confirmed through pathological anatomy. Few months later, the patient died from osseous metastasis in her spinal cord. Conclusions: early recognition of symptoms and signs of adrenal hyperfunction is very important for diagnosis and timely treatment of adrenal carcinoma(AU)

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Adrenal carcinoma is a rare malignancy of poor prognosis. The most common clinical presentation is secondary to hormone production, while the development of symptomatic hypoglycemia is exceptional. We report the case of a 37 year old-woman admitted to hospital with severe hypoglycemia, hypertension, hypokalemia and amenorrhea. In the laboratory we found hypoglycemia, with low insulin levels, and androgen levels in tumor range. CT of abdomen and pelvis showed a heterogeneous lesion of solid appearance without a cleavage plane relative to liver parenchyma, and intense contrast enhancement. Retroperitoneal mass was removed, and the patient evolved without complications, blood glucose and potassium were normalized, blood pressure stabilized and menstrual cycles recovered.

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El carcinoma suprarrenal es una neoplasia maligna infrecuente y de mal pronóstico. La presentación clínica más común es originada por la producción hormonal excesiva, mientras que el desarrollo de hipoglucemia sintomática es excepcional. Presentamos el caso de una mujer de 37 años que ingresó al hospital por síntomas de hipoglucemias graves, hipertensión arterial, hipopotasemia y amenorrea secundaria. En el laboratorio se halló hipoglucemia con insulina inhibida y niveles de andrógenos en rango tumoral. La tomografía computarizada (TC) de abdomen y pelvis mostró voluminosa formación heterogénea de aspecto sólido sin plano de clivaje con respecto al parénquima hepático e intenso realce con contraste. Luego de la extirpación de la masa retroperitoneal, evolucionó con valores de glucemia y potasemia normales, estabilizó la presión arterial y recuperó los ciclos menstruales.

Adrenal carcinoma is a rare malignancy of poor prognosis. The most common clinical presentation is secondary to hormone production, while the development of symptomatic hypoglycemia is exceptional. We report the case of a 37 year old-woman admitted to hospital with severe hypoglycemia, hypertension, hypokalemia and amenorrhea. In the laboratory we found hypoglycemia, with low insulin levels, and androgen levels in tumor range. CT of abdomen and pelvis showed a heterogeneous lesion of solid appearance without a cleavage plane relative to liver parenchyma, and intense contrast enhancement. Retroperitoneal mass was removed, and the patient evolved without complications, blood glucose and potassium were normalized, blood pressure stabilized and menstrual cycles recovered.

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El carcinoma suprarrenal es una neoplasia maligna infrecuente y de mal pronóstico. La presentación clínica más común es originada por la producción hormonal excesiva, mientras que el desarrollo de hipoglucemia sintomática es excepcional. Presentamos el caso de una mujer de 37 años que ingresó al hospital por síntomas de hipoglucemias graves, hipertensión arterial, hipopotasemia y amenorrea secundaria. En el laboratorio se halló hipoglucemia con insulina inhibida y niveles de andrógenos en rango tumoral. La tomografía computarizada (TC) de abdomen y pelvis mostró voluminosa formación heterogénea de aspecto sólido sin plano de clivaje con respecto al parénquima hepático e intenso realce con contraste. Luego de la extirpación de la masa retroperitoneal, evolucionó con valores de glucemia y potasemia normales, estabilizó la presión arterial y recuperó los ciclos menstruales.(AU)

Adrenal carcinoma is a rare malignancy of poor prognosis. The most common clinical presentation is secondary to hormone production, while the development of symptomatic hypoglycemia is exceptional. We report the case of a 37 year old-woman admitted to hospital with severe hypoglycemia, hypertension, hypokalemia and amenorrhea. In the laboratory we found hypoglycemia, with low insulin levels, and androgen levels in tumor range. CT of abdomen and pelvis showed a heterogeneous lesion of solid appearance without a cleavage plane relative to liver parenchyma, and intense contrast enhancement. Retroperitoneal mass was removed, and the patient evolved without complications, blood glucose and potassium were normalized, blood pressure stabilized and menstrual cycles recovered.(AU)

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El carcinoma suprarrenal es una enfermedad infrecuente que afecta entre 0.45 a 2 personas cada millón. Relación mujer: hombre 2.7:1, con un promedio de edad de 45 años. Mayor compromiso de la glándula izquierda. En el 53% de los casos se diagnostica por síndromes funcionantes. El resto se expresa por efecto de masa o por detección de metástasis. Son fundamentales las imágenes y el testeo hormonal. Es muy agresivo, de mal pronóstico y generalmente avanzado al diagnóstico. A la fecha existen escasos recursos terapéuticos. A continuación, se presenta una paciente de 23 años con un cáncer de suprarrenal funcionante estadio IV.

The adrenal carcinoma is an infrequent disease that affects0.45 to 2 cases per millon population per year. Women aremore frequently affected than men (2.7:1), with an averageage of 45 years. More common is the commitment of theleft gland. In 53% of cases, functioning tumor exists. Therest are diagnosed from local tumour invasion. Images andhormonal laboratory are basic. It is very aggressive, withpoor prognosis and advanced at diagnosis. To date thereare few therapeutic resources. We present here a 23 yearsold patient with such diagnosis and metastatic stage.

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