Primary malignant tumors of the adrenal glands

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

Carcinoma suprarrenal funcionante metastásico / Metastatic adrenal carcinoma functioning

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.

A Case of an Adrenocortical Carcinoma with Pulmonary Embolism as the Initial Manifestation

The annual incidence of a first episode of deep vein thrombosis or pulmonary embolism (PE) in the general population is 120 per 100,000. Cancer is associated with an approximately 4- to 7-fold higher risk of thrombosis. Adrenocortical carcinoma (ACC) is a rare type of malignancy, accounting for 0.02% of all cancers reported annually. Approximately 40% of ACCs are nonsecretory. Most patients with nonsecreting tumors have clinical manifestations related to tumor growth (e.g., abdominal or flank pain). Often the adrenal mass is detected by chance via radiographic imaging. As a result, most ACC patients are diagnosed at an advanced stage and have a poor prognosis. Herein, we report a case of a 54-year-old woman who was admitted to our emergency department complaining of dyspnea. She was diagnosed with ACC accompanied by thrombi in the pulmonary artery and inferior vena cava. We performed a left adrenalectomy and administered adjuvant radiotherapy. The patient is currently receiving warfarin and adjuvant mitotane therapy. She was incidentally diagnosed with ACC, with PE as the initial manifestation.

A Case of Adrenocortical Carcinoma Secreting Cortisol, Androgen and Aldosterone

Primary adrenocortical carcinoma is a rare tumor, and is characterized by a peri-tumor mass effect and hormone excess signs. Adrenocortical carcinoma most commonly secretes cortisol, but tumors that secrete other adrenal hormones (aldosterone, androgen) are rare. Herein, we report the case of a 70-year-old woman with cortisol, androgen, and aldosterone-secreting adrenal carcinoma. The patient complained of generalized weakness, moon face, and central obesity. On laboratory examination, hypokalemia and metabolic alkalosis was detected. On the hormone test, cortisol, DHEA-S, and aldosterone were all increased. Abdominal CT showed a large right adrenal mass. She underwent right adrenalectomy and the histology revealed the presence of an adrenocortical carcinoma. After adrenalectomy, the patient was treated with hydrocortisone and mitotane.

Pharmacological management of Cushing's syndrome: an update

The treatment of choice for Cushing's syndrome remains surgical. The role for medical therapy is twofold. Firstly it is used to control hypercortisolaemia prior to surgery to optimize patient's preoperative state and secondly, it is used where surgery has failed and radiotherapy has not taken effect. The main drugs used inhibit steroidogenesis and include metyrapone, ketoconazole, and mitotane. Drugs targeting the hypothalamic-pituitary axis have been investigated but their roles in clinical practice remain limited although PPAR-gamma agonist and somatostatin analogue som-230 (pasireotide) need further investigation. The only drug acting at the periphery targeting the glucocorticoid receptor remains Mifepristone (RU486). The management of Cushing syndrome may well involve combination therapy acting at different pathways of hypercortisolaemia but monitoring of therapy will remain a challenge.

O tratamento de escolha para a síndrome de Cushing ainda é a cirurgia. O papel da terapia medicamentosa é duplo: ele é usado para controlar o hipercortisolismo antes da cirurgia e otimizar o estado pré-operatório do paciente e, adicionalmente, quando ocorre falha cirúrgica e a radioterapia ainda não se mostrou efetiva. Os principais medicamentos são empregados para inibir a esteroidogênese e incluem: metirapona, cetoconazol e mitotano. Medicamentos visando o eixo hipotálamo-hipofisário têm sido investigados, mas seu papel na prática clínica permanece limitado, embora o agonista PPAR-gama e análogo de somatostatina, som-230 (pasireotídeo), requeira estudos adicionais. A única droga que age perifericamente no receptor glicocorticóide é a mifepristona (RU486). O manejo da síndrome de Cushing deve envolver uma combinação terapêutica atuando em diferentes vias da hipercortisolemia, mas o monitoramento dessa terapia ainda permanece um desafio.

A case of adrenal gland dependent hyperadrenocorticism with mitotane therapy in a Yorkshire terrier dog

Hyperadrenocorticism, a disorder characterized by excessive production of cortisol by the adrenal cortex, is wellrecognized in dogs. A 10-year-old, intact male, Yorkshire terrier dog was evaluated because of corneal ulceration and generalized alopecia. Diagnosis was made based on history taking, clinical signs, physical examination, and results of routine laboratory testing (complete blood count, serum biochemical analysis, and urinalysis). In addition, adrenocorticotropic hormone (ACTH) stimulation test and abdominal ultrasonography were also used to diagnose this case. The patient was diagnosed as adrenal gland neoplasia and medical therapy using the adrenocorticolytic agent, mitotane, was initiated. An ACTH stimulation test was performed after initial therapy. After successful induction was obtained, maintenance therapy with mitotane still continued.

Estrogen Producing Adrenocortical Carcinoma / 대한비뇨기과학회지

An estrogen producing adrenocortical carcinoma is a rare, highly malignant tumor. Fewer than 100 cases of feminizing adrenocortical carcinomas in men have been reported. These tumors are usually large in size and show high levels of serum total estrogen and estradiol. Recently, two cases of estrogen producing adrenocortical carcinoma were experienced by our selves during in year. A unilateral adrenalectomy, with mass excision, was performed in both patients, with an ipsilateral nephrectomy performed in just one. Mitotane or cisplatin based adjuvant chemotherapy was also performed.

Tratamiento médico de los tumores hipofisiarios productores de corticotrofina y tirotrofina / Medical treatment of adrenocorticotrophin and thyrotrophin-secreting pituitary tumors

Se analiza el estado actual del tratamiento médico de los tumores hipofisiarios productores de corticotrofina (ACTH) y tirotrofina (TSH). Se presentan las investigaciones comunicadas por diversos autores en el tratamiento de la enfermedad de Cushing y el síndrome de Nelson con moduladores de la secreción de ACTH, la bromocriptina, la ciproheptadina, la ritanserina, el ácido valproico y la somatostatina. Los resultados positivos cubren un pequeño número de casos. La primera opción de tratamiento de estos tumores es la cirugía seguida de la radioterapia. Cuando no se logra extirpar completamente los tumores, el uso de drogas que actúan inhibiendo la esteroidogénesis suprarrenal constituye una terapia paliativa. Entre estas drogas se cuentan el ketoconazol, la aminoglutetimida, la metopirona, el mitotano y el trilostan, con diversos grados de efectividad y tolerancia. Los pacientes que presentan tumores hipofisiarios productores de TSH han sido tratados con éxito con análogos de la somatostatina de acción prolongada, octreotide y lanreotide SR. Se ha logrado dramática supresión de la TSH y de la subunidad alfa, así como control del hipertiroidismo y disminución del tamaño de los tumores. Estas drogas ofrecen posibilidades de tratamiento médico para los pacientes que no logran control de su enfermedad con la cirugía

A Case of Adrenal Cortical Carcinoma with Invasion of Inferior Vena Cava / 대한내과학회지

The adrenal cortical carcinoma is a rare cancer with an estimated incidence of about 1 case per 1,700,000 population. Further development of the tumor thrombi invasion of the inferior vena cava is very rare. The adrenal cortical carcinoma has poor prognosis due to delayed onset of symptoms and signs with regional and metastatic diseases are about 70M at the time of diagnosis. The diagnosis is made by hormonal and imaging studies. Adrenal cortical carcinoma is slightly more frequent in female and hormonally non-functioning tumor is more frequent. Traditionally, surgery and mitotane chemotherapy are known as a valuable therapeutic modality. But recently usefullness of mitotane is questioned due to low response rate and complication such as gastrointestinal trouble and there is not established optimal dosage and duration of treatment. Recently, in metastatic adrenal cortical carcinoma, some literature with combined chemotherapy had tried and reported good response. But, in general, combined chemotherapy has known as ineffective. We experienced and report adrenal cortical carcinoma with inferior vena caval invasion which is diagnosed by computed tomography and magnetic resonance imaging, and we treated surgery and postoperative chemotherapy.

Carcinoma no funcionante de corteza suprarrenal: informe de un caso / Non-functional adrenal carcinoma: a case report

Los adenocarcinomas de la corteza suprarrenal son entidades clínicas muy poco comunes que alcanzan una frecuencia de 0.2 por ciento entre todas las neoplasias. Por lo regular, la mayoría de ellas son de tipo funcionante y tienen una presentación clínica que va a depender del exceso circulante de la hormona producida por cualquiera de las tres capas que componen la estructura cortical. Sin embargo, un mínimo porcentaje de estas neoplasias se consideran no funcionantes siendo, por su forma de presentación, todo un reto diagnóstico para el clínico. El objetivo del presente trabajo es informar el caso de un paciente masculino en la séptima década de la vida con pérdida de peso, masa suprarrenal derecha y otra en mesogastrio con determinación de 17 cetoesteroides, cortisol plasmático y ácido vainillilmandélico normales y en quien la necropsia mostró adenocarcinoma de corteza suprarrenal. Se realiza revisión de la literatura enfocada al diagnóstico y tratamiento

Mitotane: nuevo medicamento homeopatico en asma bronquial / Mitotane: new homeopathic medicament for asthma bronquial

En el presente estudio se presenta una revision condensada sobre el concepto actual de los dos tipos conocidos de asma: 1) extrinseca o alergica, y 2) intrinseca o idiosincrasica, ademas de los resultados en la utilizacion del farmaco mititane (o,p-DDD) como un nuevo medicamento homeopatico en el tratamiento des asma, la justificacion para su uso, su tecnica de preparacion y la casuistica de 120 pacientes de la consulta particular en el periodo de los ultimos 3 años

Carcinoma de córtex adrenal produtor de esteróides: diagnóstico e terapêutica / Adrenal cortex carcinoma producer of steroids: diagnostic and therapeutic

Os autores estudaram 24 casos de carcinoma funcionante do cørtex adrenal com o objetivo de revisar o tratamento cir£rgico e quimioter pico. Procurou-se fazer correlaüoes com o tipo de cirurgia e a efi cia da quimioterapia com a sobrevida. Observou-se que tanto a adrenalectomia como a nefroadrenalectomia ofereceram resultados semelhantes em termos de sobrevida e significativamente a sobrevida. (Arq Bras Endocrinol Metab 1994; 38/1:16-22).