Histogram analysis in the differentiation between adrenal adenomas and pheochromocytomas: the value of a single measurement / Análise por histograma para diferenciação entre adenomas da adrenal e feocromocitomas: o valor de uma medida única

Abstract Objective: To assess the diagnostic accuracy of histogram analysis on unenhanced computed tomography (CT) for differentiating between adrenal adenomas and pheochromocytomas (PCCs). Materials and Methods: We retrospectively identified patients with proven PCCs who had undergone CT examinations between January 2009 and July 2019 at one of two institutions. For each PCC, we selected one or two adenomas diagnosed within two weeks of the date of diagnosis of the PCC. For each lesion, two readers scored the size, determined the mean attenuation, and generated a voxel histogram. The 10th percentile (P10) was obtained from the conventional histogram analysis, as well as being calculated with the following formula: P10 = mean attenuation - (1.282 × standard deviation). The mean attenuation threshold, histogram analysis (observed) P10, and calculated P10 (calcP10) were compared in terms of their diagnostic accuracy. Results: We included 52 adenomas and 29 PCCs. The sensitivity, specificity, and accuracy of the mean attenuation threshold were 75.0%, 100.0%, and 82.5%, respectively, for reader 1, whereas they were 71.5%, 100.0%, and 81.5%, respectively, for reader 2. The sensitivity, specificity, and accuracy of the observed P10 and calcP10 were equal for both readers: 90.4%, 96.5%, and 92.6%, respectively, for reader 1; and 92.3%, 93.1%, and 92.6%, respectively, for reader 2. The increase in sensitivity was significant for both readers (p = 0.009 and p = 0.005, respectively). Conclusion: For differentiating between adenomas and PCCs, the histogram analysis (observed P10 and calcP10) appears to outperform the mean attenuation threshold as a diagnostic criterion.

Resumo Objetivo: Avaliar a acurácia diagnóstica da análise por histograma na tomografia computadorizada (TC) sem contraste para a diferenciação entre adenomas adrenais e feocromocitomas (FCCs). Materiais e Métodos: Identificamos, retrospectivamente, pacientes com diagnóstico de FCC confirmado que foram submetidos a exames de TC entre janeiro de 2009 e julho de 2019 em duas instituições distintas. Para cada FCC, selecionamos um ou dois adenomas diagnosticados em até duas semanas da data do diagnóstico do FCC. Para cada lesão, dois leitores pontuaram o tamanho, determinaram a atenuação média e geraram um histograma com os voxels das imagens. O percentil 10 (P10) foi obtido a partir da análise convencional do histograma, além de ser calculado com a seguinte fórmula: P10 = atenuação média - (1,282 × desvio-padrão). O limiar de atenuação média, o P10 da análise por histograma (P10 observado) e o P10 calculado (P10calc) foram comparados em termos de acurácia diagnóstica. Resultados: Foram incluídos 52 adenomas e 29 FCCs. A sensibilidade, especificidade e acurácia do limiar de atenuação média foram de 75,0%, 100,0% e 82,5% para o leitor 1, respectivamente, e de 71,5%, 100,0% e 81,5% para o leitor 2, respectivamente. A sensibilidade, especificidade e acurácia do P10 observado e do P10calc foram idênticas para os dois leitores: 90,4%, 96,5% e 92,6%, respectivamente, para o leitor 1; e 92,3%, 93,1% e 92,6%, respectivamente, para o leitor 2. O aumento da sensibilidade foi significativo para ambos os leitores (p = 0,009 e p = 0,005, respectivamente). Conclusão: Para a diferenciação entre adenomas e FCCs, a análise por histograma (P10 observado ou P10calc) parece superar o limiar de atenuação média como critério diagnóstico.

Abordaje de hipertensión secundaria en adultos mayores: reporte de caso / Secondary hypertension approach in older adults: a case report

Resumen La hipertensión arterial secundaria es poco frecuente y está asociada con una causa que puede ser tratable, por lo cual su diagnóstico y tratamiento oportuno son importantes. La orientación diagnóstica se hace con base en los datos obtenidos en la anamnesis y examen físico del paciente buscando posibles etiologías. Se presenta el caso de un hombre de 63 años, con hipertensión arterial resistente, sin otra sintomatología ni antecedentes personales o familiares. Se realizaron estudios iniciales que documentaron hipopotasemia, lo que hizo sospechar hiperaldosteronismo primario, se solicitaron pruebas complementarias con aldosterona plasmática elevada, actividad de la renina plasmática baja, además con imagen diagnóstica por resonancia nuclear magnética que mostró nódulo suprarrenal derecho. Se considero llevar a adrenalectomía derecha, con reporte de patología compatible con adenoma cortical adrenal. Durante el seguimiento en atención primaria presento adecuado control en cifras de presión arterial con disminución del número de medicamentos antihipertensivos requeridos.

Abstract Secondary arterial hypertension is rare, it is associated with a cause that can be treatable, for which its diagnosis and treatment are not important.The diagnostic orientation is made based on the data obtained in the anamnesis and physical examination of the patient, looking for possible etiologies.We present the case of a 63-year-old man with resistant hypertension, without other symptoms or personal or family history. Initial studies were performed that documented hypokalemia, which led to the suspicion of primary hyperaldosteronism. Complementary tests were requested with high plasma aldosterone, low plasma renin activity, in addition to a diagnostic magnetic resonance imaging that showed an adrenal nodule. A right adrenalectomy was considered, with a report of pathology compatible with adrenal cortical adenoma.During the follow-up in primary care, patient presented adequate control in blood pressure figures with a decrease in the number of antihypertensive drugs required.

Nursing of radiofrequency ablation for adrenocortical adenoma with Cushing's syndrome combined steroid-induced diabetes mellitus: one case report / 中国实用护理杂志

Objective:To summarize experience of nursing in radiofrequency ablation (RFA) for adrenocortical adenoma with Cushing′s syndrome combined steroid-induced diabetes mellitus.Methods:The key of preoperative nursing was precise glucose control by using insulin pump. Focus on patients psychological intervention. It was important for closely monitor the change of blood pressure, cortisol levels and biochemical index to prevent acute complications such as adrenal crisis during RFA. Pay attention to postoperative nutrition management for reduce blood glucose fluctuation and adverse reactions of hydrocortisone at postoperative.Results:No severe complications occurred during this procedure. After twenty-day nursing and treatment, the patient′s blood glucose control was stable, and the symptoms related to Cushing′s syndrome gradually disappeared.Conclusion:The ultrasound guided radiofrequency ablation in adrenocortical adenoma with Cushing′s syndrome combined with steroid-induced diabetes mellitus was a feasible, safe, and minimally invasive treatment. The key to effectively reduce postoperative complications and ensure the success of the operation. Are adequate preoperative preparation and strict monitor.

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

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

Clinical value of captopril test in noninvasive diagnosis of aldosterone-producing adenoma / 北京大学学报(医学版)

OBJECTIVE@#To analyze the clinical characteristics of aldosterone-producing adenoma (APA) subtypes in primary aldosteronism (PA) and the application value of captopril challenge test (CCT) in adenomas. And to find out the clinically specific non-invasive index for identifying APA subtypes from PA.@*METHODS@#The clinical data of hospitalized patients with hypertension were retrospectively collected. All the patients were conducted with the CCT and 90 patients with PA were confirmed. Among them, 34 patients were confirmed to have APA by surgery. The clinical indicators of the two groups of patients including plasma aldosterone concentration (PAC), aldosterone inhibition rate (%), and aldosterone to renin ratio (ARR) before and after the CCT were compared, the receiver operating characteristic (ROC) curves for the relevant indicators before and after the CCT drawn, and the areas under the curve (AUC) compared. The ROC curves were used to analyze the efficiency of the different CCT diagnostic criteria for diagnosing APA.@*RESULTS@#Compared with the PA group, the duration of hypertension was shorter, the incidence of hypokalemia was higher, and the average serum potassium level was lower when APA was diagnosed. There were no significant differences in blood pressure level, gender, serum sodium and body mass index between the two groups. Compared with PA population, APA group had higher PAC and ARR whether before or after the CCT, but lower plasma renin concentration (PRC). In APA patients, the mean degree of PAC declined after CCT was approximately 5.7%, but 5% with that of PA. As for diagnosing, ARR before or after CCT had diagnostic value for APA, in which the ARR cut-off point was 7.12, which yielded a sensitivity and specificity of 35.85% and 77.78%. The cut-off point of ARR after CCT was 4.23, with a sensitivity of 71.43% and specificity of 62.22%. For the diagnosis, the ARR before and after CCT were of no significant difference. However, the diagnostic specificity of ARR>7.12 combined with hypokalemia was up to 80%.@*CONCLUSION@#ARR before or after CCT have clinical value for the diagnosis of APA from PA, when combined with hypokalemia yielded high specificity.

Assessment of Subclinical Cardiac Alterations and Atrial Electromechanical Delay by Tissue Doppler Echocardiography in Patients with Nonfunctioning Adrenal Incidentaloma / Avaliação de Alterações Cardíacas Subclínicas e Atraso Eletromecânico Atrial por Ecocardiografia com Doppler Tecidual em Pacientes com Incidentaloma Adrenal Não Funcionante

Abstract Background: Majority of the incidentally discovered adrenal masses, called adrenal incidentaloma (AI), are nonfunctioning adrenal adenomas. The appropriate management of AI is still a matter debate, so it is necessary to investigate their associated morbidity. However, data regarding morphological and functional cardiac alterations are limited in this group. Objective: In this study, we aimed to assess cardiac structural and functional characteristics and atrial conduction properties in patients with nonfunctioning AI. Methods: Thirty patients with nonfunctioning AI and 46 properly matched control subjects were included in the study. After hormonal and biochemical analysis, all participants underwent transthoracic echocardiography to obtain systolic and diastolic parameters of both ventricles, in addition to atrial conduction times by tissue Doppler echocardiography. Data were analyzed with Statistical Package for the Social Sciences (SPSS, Chicago, IL, United States) statistics, version 17.0 for Windows. P < 0.05 was considered statistically significant. Results: Left ventricular (LV) mass index and LV myocardial performance index were significantly increased in AI group. Among atrial conduction times, both intra- and interatrial electromechanical delays were significantly prolonged in patients with nonfunctioning AI. Other laboratory and echocardiographic findings were similar between groups. Conclusion: Our study revealed that intra- and inter-atrial conduction times were prolonged, and LV mass index was increased in patients with nonfunctioning AI. These findings may be markers of subclinical cardiac involvement and tendency to cardiovascular complications. Close follow-up is necessary for individuals with nonfunctioning AI for their increased cardiovascular risk.

Resumo Fundamento: A maioria das massas adrenais descobertas incidentalmente, denominadas incidentaloma adrenal (IA), são adenomas adrenais não funcionantes. O manejo adequado da IA ainda é um tema de debate, e por isso é necessário investigar suas morbidades associadas. Entretanto, dados referentes a alterações cardíacas morfológicas e funcionais são limitados nesse grupo. Objetivo: Neste estudo, objetivamos avaliar as características estruturais e funcionais cardíacas e as propriedades de condução atrial em pacientes com IA não funcionante. Métodos: Trinta pacientes com IA não funcionante e 46 controles adequadamente pareados foram incluídos no estudo. Após análise hormonal e bioquímica, todos os participantes foram submetidos a ecocardiograma transtorácico para obtenção de parâmetros sistólicos e diastólicos de ambos os ventrículos, além dos tempos de condução atrial pelo ecocardiograma com Doppler tecidual. Os dados foram analisados com o Statistical Package for the Social Sciences (SPSS, Chicago, IL, Estados Unidos), versão 17.0 para Windows. P < 0,05 foi considerado estatisticamente significativo. Resultados: O índice de massa do ventrículo esquerdo (VE) e o índice de desempenho miocárdico do VE foram significativamente aumentados no grupo IA. Entre os tempos de condução atrial, os atrasos eletromecânicos intra- e interatriais foram significativamente prolongados em pacientes com IA não funcionante. Outros achados laboratoriais e ecocardiográficos foram semelhantes entre os grupos. Conclusão: Nosso estudo revelou que os tempos de condução intra- e interatrial estavam prolongados e o índice de massa do VE estava aumentado em pacientes com IA não funcionante. Esses achados podem ser marcadores de envolvimento cardíaco subclínico e de tendência a complicações cardiovasculares. Um acompanhamento rigoroso é necessário para indivíduos com IA não funcionante, devido ao aumento do risco cardiovascular.

Clinicopathologic characteristics and reticular fiber staining of adrenocortical carcinoma / 临床与实验病理学杂志

Purpose To analyse the clinicopathologic characteristics and reticular fiber staining of adrenocortical carcinoma (ACC).Methods The clinical,imaging,pathological data and reticular fiber staining of 20 cases with adrenocortical adenoma (ACA) and 16 cases with ACC were analyzed retrospectively.Results The median course of disease and maximum diameter of ACA were 21 months and 2.6 cm respectively,but ACC were 5 months and 9.5 cm respectively.9 cases with ACC were diagnosed with distant metastasis.Histologically,ACA cells arranged in nest-like and coarse trabecular forms with acidophilic or clear cytoplasm,unconspicuous nuclear atypia,and rare mitotic figures.The capsules,sinusoids and veins were uninvolved,and hemorrhage,necrosis and thick collagen fibers were infrequent.The Weiss score of all ACA was less than 2 (average =0.85 ± 0.81).ACC cells arranged in nest-like,coarse trabecular,cord-like,and diffuse structure with acidophilic cytoplasm,various nuclear atypia and atypical mitotic figures.The invasion of capsules,sinusoids and veins,hemorrhage and necrosis regions and thick collagen fibers were easily found.The Weiss score of all ACC was more than 3 (average =5.2 ±1.3).Immunohistochemical staining showed that vimentin,Syn,NSE,CK,CR,Melan-A and α-inhibin were expressed similarly between ACA and carcinoma (P ＞ 0.05).Reticular fiber staining revealed that the reticular fiber structures of ACA were unbroken,but ACC were destructed with various degrees of rupture,collapse,sparsity or disappearance (P ＜ 0.01).Conclusion ACC lacks characteristic immune phenotype,but increased diameter,distant metastasis,thick collagen fibers and destructed reticular fibrillar network are helpful in the diagnosis of ACC.

Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders

OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

Exploration of the optimal diameter cut-off value in patients with nonfunctional adrenal tumor suitable for surgery / 解放军医学杂志

Objective To analyze the pathology of the patients with nonfunctional adrenal tumor (NFA), and explore the optimal diameter cut-off value. Methods The clinical data of 243 patients with NFA, evaluated in the Department of Endocrinology and operated in the Department of Urology of General Hospital of Chinese PLA from Feb. 1996 to Jan. 2016, were collected. The patients were divided into two groups according to pathology: those in real demand of surgery were classified to the surgery-need group (n=S7), while the others were categorized as the surgery-unwanted group (n=186). The general situation, pathological type and tumor diameter of the two groups and the factors affecting the surgery were analyzed, and the ROC curve was used to explore the optimal surgery cut-off value, which represents the maximum value of the sum of sensitivity and specificity. Results Of the 57 patients in surgery-need group (27 males and 30 females), the lesions were on the right in 31 cases, on the left in 25 cases, and on bilateral sides in 1 case; the median of lesion diameter was 4.5cm, and the average age was 41.5 ± 12.1 years old. Of the 186 patients in surgery-unwanted group (87 males and 99 females), the lesions were on the right in 99 cases, on the left in 86 cases, and on bilateral sides in 1 case; the median of lesion diameter was 3.0cm, and the average age was 50.6 ± 10.9 years old. Logistic regression revealed that lesion diameter might be a risk factor (OR= 1.340, 95%CI 1.266-1.418, P=0.000) and age be a protective factor (OR=0.942, 95%CI 0.929-0.955, P=0.000) for real demand of surgery. The area under the ROC curve (AUC) of lesion diameter was 0.757(95%CI 0.681-0.833). The optimal cut-off value was 4.1cm (sensitivity 60.7% and specificity 83.0%). Conclusions Younger patients with bigger lesion diameter may have greater possibility for surgery. The optimal surgery cut-off value of the lesion diameter is 4.1cm.

Diagnosis and Treatment of Adrenal Black Adenoma：Report of 7 Cases / 中国微创外科杂志

Objective To explore the diagnosis and treatment of adrenal black adenoma . Methods From June 2002 to July 2014, 7 patients with adrenal tumors were treated with retroperitoneal laparoscopic partial adrenalectomy in our hospital .During the operation all the patients were placed in healthy lateral decubitus position .Three trocars were introduced into the lumbar region:below the 12th rib along the anterior and posterior axillary lines , and 2 cm beyond superior iliac spine on the middle axillary line .The retroperitoneal space was established with homemade balloon-expanding devices .Afterwards , the tumors were removed by using a harmonic scalpel. Results All the tumors were resected completely .The average operation time was 60 min (range, 34-90 min) and the average blood loss in the operation was 70 ml (range, 20 -200 ml) without blood transfusion.The drainage tubes were removed on the 3rd or 4th postoperative day and the patients were discharged on the 6th or 7th postoperative day .They were pathologically diagnosed as adrenal black adenoma . Two patients manifested the Cushing ’ s syndrome and 3 patients showed hypertension , all of which were recovered to normal postoperatively .No recurrence or metastasis was noted during a follow-up for 12-24 months (mean, 18 months). Conclusions Adrenal black adenoma, without specific clinical features, is a rare kind of adrenocortical adenoma .The confirmative diagnosis depends on pathological results .Retroperitoneal laparoscopic surgery is a minimally invasive, safe and effective technique for adrenal black adenoma , which provides favorable prognosis .

Consumption of an Excessive Amount of Ionic Beverage Can Trigger Adrenal Adenoma - Induced Severe Hypokalemic Rhabdomyolysis / 대한내과학회지

Rhabdomyolysis results from acute damage to skeletal muscles caused by various conditions, of which hypokalemia is a recognized but rare example. Although primary aldosteronism may cause severe hypokalemia leading to rhabdomyolysis, the potassium level of such patients can be within the normal range. Hypokalemia is most frequently triggered when these patients are exposed to an additional insult, such as diuretic stress. Similarly, overzealous consumption of ionic beverages with osmotic diuretic effects can cause hypokalemia. Here, we describe a patient with an aldosterone-secreting adrenal adenoma, who presented with acute rhabdomyolysis secondary to severe hypokalemia triggered by consumption of a large volume of ionic beverage for 3 weeks.

17α-hydroxylase Deficiency Mimicking Hyperaldosteronism by Aldosterone-producing Adrenal Adenoma / 대한내과학회지

17α-hydroxylase deficiency is a rare cause of congenital adrenal hyperplasia and is characterized by primary amenorrhea, delayed puberty and hypertension. Although 17α-hydroxylase deficiency mimics mineralocorticoid-induced hypertension, impaired sexual development can aid in the differential diagnosis of this disease. A 32-year-old woman, who had a history of testicular feminization syndrome, presented with hypertension. Her aldosterone level was elevated whereas plasma renin activity was reduced, and her computed tomography scan showed a left adrenal adenoma, which was thought to be an aldosterone producing adenoma. A left adrenalectomy was performed to treat hypertension; however, the condition did not improve. The hormonal tests revealed high levels of plasma progesterone, mineralocorticoid and adrenocorticotropic hormone, and low levels of 17a hydroxyprogesterone, cortisol and sex hormones. The patient was diagnosed with 17α-hydroxylase deficiency and commenced on prednisolone, which controlled hypertension. Here, we report a case of 17α-hydroxylase deficiency mimicking hyperaldosteronism via aldosterone-producing adrenal adenoma.

Tumores e hiperplasias adrenocorticais: identificação de possíveisdoenças hereditárias / Recognition of hereditary diseases related to adrenocortical tumors andhyperplasias

A alta prevalência de tumores da glândula adrenal deve-se, em parte, ao avanço dos métodos de imagem. Os adenomas, carcinomas e hiperplasias oriundos do córtex adrenal são responsáveis por 80 a 90% dos processos tumorais. Alguns casos são herdados e podem estar associados a efeito compressivo de massa tumoral, hipersecreção de esteroides ou manifestações clínicas em outros órgãos. Considerando as hiperplasias e tumores adrenocorticais, o objetivo desse trabalho foi auxiliar os médicos na identificação de pacientes que apresentem risco para doença hereditária. As neoplasias e hiperplasias adrenocorticais podem ser encontradas em síndromes hereditárias, como a síndrome de Li-Fraumeni, síndrome de Beckwith-Wiedemann, neoplasia endócrina múltipla do tipo I, síndrome de Gardner e no complexo de Carney. A hereditariedade também está associada com doenças adrenocorticais na hiperplasia adrenal congênita, no aldosteronismo primário e/ou na síndrome de Cushing (doença clínica ou subclínica) na hiperplasia adrenal macronodular primária. Essa revisão descreve as características clínicas e os defeitos genéticos responsáveis pelas síndromes hereditárias. Relacionamos também a classificação histopatológica dos processos expansivos com os principais sinais clínicos e os genes relacionados. A identificação de defeitos genéticos em células germinativas nessas doenças familiais permite o conhecimento de alterações somáticas em alguns tipos de processos tumorais adrenocorticais de etiologia esporádica. Considerando a prevalência dos tumores do córtex adrenal, a identificação de predisposição hereditária é essencial para assegurar a conduta clínica correta do paciente e o aconselhamento genético de seus familiares.

The adrenal gland tumors are prevalent due in part by the widespread use of imaging studies. Adenomas, carcinomas and hyperplasias, originating from the adrenal cortex, account for 80-90% of adrenal tumoral processes. Some cases are inherited and may be associated with local mass effect, steroid hypersecretion and/or clinical manifestation in other organs. In the context of adrenocortical tumors and hyperplasias, the purpose of this article is to assist physicians in identifying patients who may be at risk of hereditary diseases. Adrenocortical hyperplasias and neoplasias can be found in familial tumor syndromes, such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia type 1, Gardner syndrome and Carney complex. Heredity has been also associated with adrenocortical lesions in congenital adrenal hyperplasia, primary aldosteronism and/or Cushing syndrome (overt or subclinical disease) in primary macronodular adrenal hyperplasia (PMAH). This review describes the clinical recognition and genetic defects that have been found to be responsible for these hereditary diseases. Furthermore, we present the histopathologic classification of adrenocortical expansive processes in correlation to the main clinical features and related genes. The identification of germline genetic defects in such familial diseases lead to the identification of somatic alterations in a subgroup of sporadic adrenocortical lesions. Considering the prevalence of adrenocortical tumors, identification of a hereditary predisposition is essential to assure the adequate clinical management of the patient and to offer the genetic counselling to family members.

Hiperaldosteronismo primário, como descrito por Conn. Relato decaso / Hyperaldosteronism, as described by Conn. Case report

O hiperaldosteronismo primário é causa de hipertensão arterialsecundária, com possibilidade de cura após cirurgia em 30a 75% dos casos. O objetivo deste estudo foi relatar um casode hipertensão arterial secundária a adenoma adrenal produtorde aldosterona. Paciente feminino, 35 anos, natural de Beruri(AM), procedente de Manaus, foi admitida com pressão arterialde 220x125mmHg associada a fraqueza muscular de membrosinferiores e câimbras. Na história patológica pregressa, havia orelato de hipertensão arterial diagnosticada há 2 anos, em tratamentocom três classes de anti-hipertensivos. Durante internaçãoem hospital geral, o quadro relatado associado à hipocalemiae alcalose metabólica sugeriram diagnóstico de hipertensão arterialsecundária a hiperaldosteronismo primário. Níveis elevadosde aldosterona plasmática, com renina suprimida e relação aldosterona-renina elevada, confirmaram o diagnóstico de hiperaldosteronismoprimário. A tomografia computadorizada deabdome evidenciou lesão tumoral com 2,3x2,0cm em glândulasuprarrenal esquerda sugestiva de adenoma adrenal. Pacientefoi submetida a adrenalectomia à esquerda com histopatologiacompatível com adenoma adrenal. Seis meses após a cirurgia,paciente evoluiu com normalização da calemia, porém mantevehipertensão arterial com necessidade de terapia anti-hipertensiva,sem novos picos hipertensivos. Se hiperaldosteronismo primáriodiagnosticado precocemente, há possibilidade de cura dahipertensão arterial secundária após adrenalectomia, reduzindo o efeito deletério da mesma sobre os sistemas cardiovascular, cerebrovasculare renal.(AU)

The primary hyperaldosteronism is a cause of secondaryhypertension, with the possibility of healing after surgery in 30to 75% of cases. The objective of this study was to report a caseof secondary hypertension to aldosterone-producing adrenaladenoma. A female patient, 35 years, from Beruri (AM), Brazil,who came to Manaus, was admitted into the emergency room withblood pressure of 220x125mmHg, associated with lower limbmuscle weakness and cramps. In the past medical history, there wasa report of hypertension diagnosed 2 years before, and she was undertreatment with three classes of antihypertensive drugs. Duringhospitalization in a general hospital, the case reported associatedto hypokalemia and metabolic alkalosis suggested a diagnosis ofhypertension secondary to primary hyperaldosteronism. Elevatedlevels of plasmatic aldosterone, suppressed renin and highaldosterone-renin relation suggested primary hyperaldosteronism.The abdominal computerized tomography scan revealed a tumor of2.3x2.0cm in the left adrenal gland, suggesting adrenal adenoma.Patient underwent left adrenalectomy and histopathology wascompatible to adrenal adenoma. After 6 months of surgery, thepatient experienced normalization of kalemia but kept arterialhypertension, requiring anti-hypertensive therapy, but had no othershypertensive peaks. Early diagnosis of primary hyperaldosteronismallowed the cure of hypertension after adrenalectomy, reducingdeleterious effects of high blood pressure levels on the cardiovascular,cerebrovascular and renal systems.(AU)

Prevalence and postoperative changes of metabolic syndrome in patients with adrenocortical adenoma / 中国糖尿病杂志

Objective To investigate changes of metabolic syndrome (MS ) in patients with adrenocortical adenoma (ACA ). Methods Sixty‐five patients with aldosterone producing adenoma (APA) ,43 patients with cortisol‐secreting adenoma (CSA) and 24 patients with nonfunctional adrenal adenoma (NAA) were evaluated. Body Mass Index (BMI) ,blood pressure ,plasma lipid ,fasting and 2 h postprandial plasma glucose ,plasma and urinary aldosterone ,plasma potassium ,basic and upright plasma renin activity (PRA ) ,plasma and urinary cortisol ,plasma ACTH and plasma potassium ACA were examined and compared with those from 53 patients of essential hypertension (EH ) and 58 controls. Adrenalectomy was performed for all the patients with adrenocortical adenoma. The data after operation were compared with those before operation. Results (1) The prevalence of MS was 38.5% ,41.9% , 20.8% ,24.5% and 17.5% in the groups respectively. The prevalence of obesity/overweight was higher in CSA group than in the other groups. There were 44.6% patients with hyperglycemia in APA group ,and 48.8% in CSA group. The prevalence of hypertension was the highest in APA and EH group ,followed by CSA group ,NAA and control groups. There were 55.4% patients with lipid disturbance in APA group , and 46.5% in CSA group. (2 ) We divided all APA and CSA patients into groups with or without MS. The APA patients with MS had higher plasma aldosterone than those without MS. The CSA patients with MS had higher 08 :00 and 24 :00 plasma cortisol than those without MS. (3) After operation ,the plasma and urinary aldosterone were decreased and the plasma potassium was increased in APA patients. The 08 :00 ,24 :00 plasma cortisol and urinary cortisol were decreased and the 08 :00 plasma ACTH was increased in CSA patients. There were no significant differences in hormonal measurements of NAA patients before and after operation. (4 ) The prevalence of MS was 27.7% ,27.9% and 29.2% in three groups after operation. The systolic and diastolic blood pressures were decreased in APA patients after operation ,and the systolic blood pressure decreased in CSA patients. There was no significant difference in MS measurements of NAA patients before and after operation. Conclusions Our study indicates that patients with functional adrenocortical adenoma have a significantly higher prevalence of metabolic syndrome which is related to over secretion of adrenal cortex hormone. After operation ,accompanying normalization of hormonal secretion ,the condition of MS in functional adrenocortical adenoma patients is improved.

Untreated Congenital Adrenal Hyperplasia with 17-alpha Hydroxylase/17,20-Lyase Deficiency Presenting as Massive Adrenocortical Tumor

Congenital adrenal hyperplasia (CAH) with 17alpha-hydroxylase/17,20-lyase deficiency is usually characterized by hypertension and primary amenorrhea, sexual infantilism in women, and pseudohermaphroditism in men. hypertension, and sexual infantilism in women and pseudohermaphroditism in men. In rare cases, a huge adrenal gland tumor can present as a clinical manifestation in untreated CAH. Adrenal cortical adenoma is an even more rare phenotype in CAH with 17alpha-hydroxylase/17,20-lyase deficiency. A 36-year-old female presented with hypertension and abdominal pain caused by a huge adrenal mass. Due to mass size and symptoms, left adrenalectomy was performed. After adrenalectomy, blood pressure remained high. Based on hormonal and genetic evaluation, the patient was diagnosed as CAH with 17alpha-hydroxylase/17,20-lyase deficiency. The possibility of a tumorous change in the adrenal gland due to untreated CAH should be considered. It is important that untreated CAH not be misdiagnosed as primary adrenal tumor as these conditions require different treatments. Adequate suppression of adrenocorticotropic hormone (ACTH) in CAH is also important to treat and to prevent the tumorous changes in the adrenal gland. Herein, we report a case of untreated CAH with 17alpha-hydroxylase/17,20-lyase deficiency presenting with large adrenal cortical adenoma and discuss the progression of adrenal gland hyperplasia due to inappropriate suppression of ACTH secretion.

A Case of a Coexisting Aldosterone-Producing Adrenal Adenoma and Ipsilateral Renal Artery Stenosis / 대한내과학회지

The simultaneous occurrence of renovascular hypertension and an aldosterone-producing adrenal adenoma is a rare entity. Here, we report the case of a 52-year-old female who had a coexisting aldosterone-producing adrenal adenoma and ipsilateral renal artery stenosis. She was diagnosed with the aldosterone-producing adrenal adenoma and then underwent a laparoscopic left adrenalectomy. Her blood pressure was uncontrolled after the adrenalectomy. Selective renal angiography showed left renal artery stenosis; thus, she underwent balloon angioplasty at the same sitting. Subsequently, her blood pressure returned to normal after administration of a single antihypertensive drug. This case suggests that it is important to recognize the possible coexistence of renal artery stenosis in a patient with an aldosterone-producing adrenal adenoma.

Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently

A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses.

Ultrasonic manifestations of large adrenal cortical adenoma / 中华医学超声杂志（电子版）

Objective To analyze the sonographic ifndings of large adrenal cortical adenoma. Methods The sonographic characteristics of thirteen cases of large adrenal cortical adenoma (diameter≥4.0 cm) which were diagnosed by surgery from January 2006 to August 2012 were retrospectively reviewed. Results In all cases, the adenomas had distinct margins. The mean size of lesions was (5.5±1.6) cm, ranging from 4.0 cm to 8.8 cm. On echotexture, three lesions were homogeneous and the remaining ten lesions were heterogeneous. In addition, seven contained hypoechoic nodules and hyperechoic septum, three had anechoic area, three had extremely hyperechoic area with acoustic shadow, and two had patchy hyperechoic area. On Doppler, most lesions had no lfow signal. Conclusions Large adrenal adenomas have complete capsule and heterogeneous internal echoes with septum, calciifcation, necrosis and hemorrhages. These characteristics are less frequently seen incommon adrenal adenoma, therefore may be helpful in recognition of large adrenal adenoma in clinical practice.

Myxoid Adrenocortical Adenoma: Magnetic Resonance Imaging and Pathology Correlation

We report a case of a 74-year-old female with myxoid adrenocortical adenoma which showed different magnetic resonance imaging findings compared to those of a typical adrenocortical adenoma. The myxoid change in the adrenocortical adenoma is a rare form of degeneration. It presents a considerable diagnostic challenge to both radiologists and clinicians because it can mimic other adrenal tumor types on imaging. The MRI findings of the presented case included a high signal intensity on T2-weighted images similar to that of fluid and delayed progressive enhancement.