The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Extensive rhabdoid morphology in ACC has been described recently in very few cases. The proportion of rhabdoid morphology and the role of SMARCB1/ INI1 expression in these tumor cells to diagnose the specific variant is not described in the literature. We reviewed the clinicopathological features of nine cases of adrenocortical neoplasm. Out of which, three cases of ACC showed predominant rhabdoid morphology. Large discohesive cells with abundant cytoplasm containing eosinophilic inclusions, eccentric vesicular nucleus, and prominent nucleoli. INI1 immunostain was retained in all cases. We reported the rhabdoid variant of ACC, a novel entity, and its diagnostic approach from their histological mimickers. Identifying more cases of this entity will help to clearly understand the pathogenesis, biologic behaviour, and any specific molecular alterations in the future.

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.

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.

Aldosterone and cortisol-secreting adrenocortical carcinoma: a 10-year follow-up case report and literature review / 中华内分泌代谢杂志

A 10-year follow-up case of aldosterone and cortisol-secreting adrenocortical carcinoma, a rare disease presenting as an adrenal space-occupying lesion accompanied with hypertension and hypokalemia was reported, and the medical record was investigated in detail. Through case presentation and literature review, it was noted that patients with adrenocortical carcinoma presented a difference in clinical manifestations, pathology, and biological behaviors. This paper may help clinicians enhance their understanding of adrenocortical carcinoma. Especially significant adrenal space-occupying lesions highly suspicious of recurrence and producing different types of endocrine hormones should be paid more attention.

Surgical strategy and procedure of adrenocortical carcinoma with retrohepatic inferior vena cava tumor thrombus with intraoperative ultrasound guidance / 中华肝胆外科杂志

Adrenocortical carcinoma (ACC) with inferior vena cava thrombosis is rare and has a poor prognosis, and the current literature overwhelmingly supports aggressive surgical intervention. This article summarizes the management of a patient with ACC with inferior vena cava thrombosis, and discusses the feasibility of detailed preoperative imaging data and intraoperative ultrasound to assess the superior and inferior boundaries of ACC with inferior vena cava thrombosis, while describing the intraoperative ultrasound-guided surgical planning and procedure for ACC with retrohepatic inferior vena cava tumor thrombus. Furthermore, it also demonstrates that it is feasible to accurately assess the superior and inferior boundaries of ACC with inferior vena cava thrombosis by preoperative multimodal imaging and intraoperative ultrasound, determine the mode of flow blockage during the operation, and obtain radical resection of the tumor.

One case report of adrenal crisis with difficulty in weaning after lumbar spinal canal decompression surgery / 中华内分泌外科杂志

Adrenocortical crisis (AC) is a kind of endocrine emergency, often occurs in infection, shock, trauma, or postoperative, if the processing is not handling timely, can endanger patient's life.But as the disease is not common and the clinical symptoms are not typical,so it is easy to be misdiagnosis and missed diagnosis.This case was a "lumbar spinal canal decompression surgery" patient, who appeared postoperative confusion, oxygenation decline,and could not seperated from breathing machine, clinical manifestations were atypical.

Diagnostic and prognostic utility of SF-1 in adrenal cortical tumours

Background and Aims: Superior imaging techniques have increased the recognition of adrenal pathology. Distinguishing benign from malignant adrenocortical tumors is not always easy. Several criteria and immunohistochemical markers have been discovered which help to differentiate between adrenocortical adenoma (ACA) and adrenocortical carcinoma (ACC). Our aim here was to evaluate the diagnostic and prognostic role of steroidogenic factor-1 (SF-1) in adult adrenocortical tumors (ACT) diagnosed using the Weiss criteria. In this cohort, we have also analyzed Ki67 and p53 expression and the extent of agreement between SF-1 and Ki-67. Methodology: This was a retrospective, observational study comprising 24 cases of adult ACT over 10 years. Immunohistochemical staining for SF-1, Ki67, and p53 was done in all the cases, and the results correlated with the morphological diagnosis made using Weiss criteria. Results: SF-1 was 100% sensitive and 80% specific as a marker of malignancy. Increased SF-1 expression correlated with worse survival. There was a moderate degree of agreement between Ki-67 labeling-index and SF-1 as a marker of malignancy with the kappa coefficient being 0.75. The sensitivity of p53 was lower than Ki67 in diagnosing ACC. Conclusion: In adult ACTs, SF-1 has diagnostic significance and prognostic implication. SF-1 is a crucial, dosage-dependent survival factor in ACC. There is a moderate extent of agreement between Ki-67 and SF-1 as a marker of malignancy.

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.

Carcinoma corticosuprarrenal virilizante en un niño / Virilizing adrenocortical carcinoma in a child

Introducción: Los tumores suprarrenales en niños son poco frecuentes y el carcinoma suprarrenal representa menos de un 10 %. En el prepúber, la manifestación más típica es el desarrollo de pubertad precoz. Objetivo: Describir las características clínicas, los procederes diagnósticos y terapéuticos de un paciente con carcinoma adrenal en edad pediátrica. Presentación de caso: Paciente de 8 años, masculino y de piel blanca con antecedentes de salud. Acude a la consulta por crecimiento de vello pubiano y aumento del pene en longitud y grosor de aproximadamente 2 años de evolución. En el examen físico se constatan aumento de la velocidad de crecimiento y signos sugestivos de virilización (voz gruesa, vello axilar, vello sexual púbico y genitales externos estadio III de Tanner). Se realizaron estudios hormonales que corroboraron el hiperandrogenismo por secreción endógena autónoma, con niveles de gonadotropinas suprimidas, niveles de testosterona y dehidroepiandrosterona elevados. También se realizaron estudios imagenológicos que evidenciaron edad ósea acelerada y la existencia de un tumor. Se realizó una adrenalectomía izquierda y se confirmó por anatomía patológica el carcinoma corticosuprarrenal virilizante izquierdo en estadío 2. Inició un tratamiento con quimioterapia por dicho diagnóstico y actualmente se mantiene en seguimiento. Conclusiones: Los carcinomas corticosuprarrenales en niños son mayoritariamente funcionantes y constituyen una de las causas de pubertad precoz periférica. Estos son infrecuentes y agresivos, por lo que la realización de estudios genéticos en familias con síndromes hereditarios contribuiría a su diagnóstico precoz para un adecuado tratamiento y mejor pronóstico(AU)

Introduction: Adrenal tumors in children are rare and adrenal carcinoma represents less than the 10 percent. In the prepubescent, the most typical manifestation is the development of early puberty. Objective: Describe the clinical characteristics and diagnostic and therapeutic procedures of a patient with adrenal carcinoma in a pediatric age. Case presentation: 8-year-old male, white-skinned patient with a history of health conditions. He attentds to the consultation due to pubic hair growth and penis enlargement in length and thickness of approximately 2 years of evolution. Physical examination shows increased growth rate and signs suggestive to virilization (deep voice, axillary hair, pubic sexual hair and external genitalia in Tanner's stage III). Hormonal studies were carried out that corroborated hyperandrogenism by autonomic endogenous secretion, with suppressed gonadotropin levels, elevated testosterone and dehydroepiandrosterone levels. Imaging studies were also performed that showed accelerated bone age and the existence of a tumor. A left adrenalectomy was performed and stage 2 left virilizing adrenocrotical carcinoma was confirmed by pathological anatomy studies. He began chemotherapy treatment for this diagnosis and is currently being followed up. Conclusions: Adrenocortical carcinomas in children are mostly functioning and are one of the causes of peripheral early puberty. These are uncommon and aggressive, so genetic studies in families with hereditary syndromes would contribute to their early diagnosis for adequate treatment and better prognosis(AU)

Classic and current concepts in adrenal steroidogenesis: a reappraisal

ABSTRACT Adrenal steroid biosynthesis and its related pathology are constant evolving disciplines. In this paper, we review classic and current concepts of adrenal steroidogenesis, plus control mechanisms of steroid pathways, distribution of unique enzymes and cofactors, and major steroid families. We highlight the presence of a "mineralocorticoid (MC) pathway of zona fasciculata (ZF)", where most circulating corticosterone and deoxycorticosterone (DOC) originate together with 18OHDOC, under ACTH control, a claim based on functional studies in normal subjects and in patients with 11β-, and 17α-hydroxylase deficiencies. We emphasize key differences between CYP11B1 (11β-hydroxylase) and CYP11B2 (aldosterone synthase) and the onset of a hybrid enzyme - CYP11B1/CYP11B2 -, responsible for aldosterone formation in ZF under ACTH control, in "type I familial hyperaldosteronism" (dexamethasone suppressible). In "apparent MC excess syndrome", peripheral conversion of cortisol to cortisone is impaired by lack of 11β-hydroxysteroid dehydrogenase type 2, permitting free cortisol access to MC receptors resulting in severe hypertension. We discuss two novel conditions involving the synthesis of adrenal androgens: the "backdoor pathway", through which dihydrotestosterone is formed directly from androsterone, being relevant for the fetoplacental setting and sexual differentiation of male fetuses, and the rediscovery of C19 11-oxygenated steroids (11-hydroxyandrostenedione and 11-ketotestosterone), active androgens and important markers of virilization in 21-hydroxylase deficiency and polycystic ovaries syndrome. Finally, we underline two enzyme cofactor deficiencies: cytochrome P450 oxidoreductase which partially affects 21- and 17α-hydroxylation, producing a combined clinical/hormonal picture and causing typical skeletal malformations (Antley-Bixler syndrome), and PAPSS2, coupled to SULT2A1, that promotes sulfation of DHEA to DHEAS, preventing active androgens to accumulate. Its deficiency results in reduced DHEAS and elevated DHEA and androgens with virilization. Future and necessary studies will shed light on remaining issues and questions on adrenal steroidogenesis.

Carcinoma de corteza suprarrenal. Presentación de un caso y revisión de la literatura

RESUMEN El carcinoma suprarrenal es un tumor raro pero devastador, esto se debe fundamentalmente a que en la mayoría de los casos se encuentra en estadios avanzados en el momento del diagnóstico. Tiene una incidencia de 0,5-2 casos por un millón habitantes al año. Entre el 40 y el 70 % de los pacientes tienen metástasis cuando se inicia el estudio. Se presenta de manera general en adultos, aunque también afecta a los niños. La mediana de edad en el momento del diagnóstico es 46 años, siendo más frecuente en el sexo masculino. Se presenta el caso de una paciente con antecedentes de hipertensión arterial de un año de evolución, ante los síntomas presentados se le realizan estudios por imágenes y de laboratorio diagnosticándose un tumor suprarrenal izquierdo. Se le realizó supraadrenalectomía izquierda obteniéndose como resultado histopatológico un carcinoma adenocortical suprarrenal. Se presenta este caso porque el carcinoma suprarrenal representa una entidad poco frecuente y es relevante exponer esta experiencia para el manejo de este tipo de neoplasia.

ABSTRACT Adrenal carcinoma is a rare devastating tumor, mainly because in most cases it is in advanced stages at the time of diagnosis. It has an incidence of 0.5-2 cases per one million inhabitants per year, in which 40 to 70 % of patients have metastases when the study begins. It occurs mainly in adults, although it also affects children. The median age at the time of diagnosis is 46 years, being more frequent in males. The case of a patient with a history of arterial hypertension of one year of evolution is presented. In view of the symptoms presented, imaging and laboratory studies are performed, diagnosing a left adrenal tumor. A left supraadrenelectomy was performed, obtaining an adenocortical adrenal carcinoma as a histopathological result.

Giant virilising adrenal cortical carcinoma

Androgen secreting adrenocortical carcinoma (ACC) is a very rare disease with a poor prognosis. Approximately 80% of tumors are functional, most commonly secreting glucocorticoids. We herewith report a case of a huge functional ACC of the right adrenal gland in a 33-year-old female who presented with complaints of hirsutism, amenorrhea and an abdominal lump. On abdominal examination a large lump was palpable in the right hypochondrium reaching up to the umbilicus. Contrast-enhance computed tomography (CECT) revealed a mass in the right suprarenal region. The tumor measured 29 cm × 20 cm × 12 cm and weighed 7.8 kg, the largest reported case of ACC in the world to the best of our knowledge.

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.

Construction and verification of tumor microenvironment-related gene prognostic model for adrenocortical carcinoma / 肿瘤研究与临床

Objective:Bioinformatics method was used to screen out prognostic model constructed by the tumor microenvironment (TME)- related genes of adrenocortical carcinoma (ACC), and the prognostic model was verified to provide clinical guidances and related biomarkers for the diagnosis and treatment of ACC.Methods:Transcriptome and clinicopathological data of 79 ACC patients were collected from the Cancer Genome Atlas (TCGA) database. ESTIMATE algorithm was used to calculate immune score, stromal score (both reflect TME) and ESTIMATE score; VennDiagram was used to select differentially expressed genes among immune score, high and low stromal score groups (grouped by median value); Gene Ontology (GO) database and Kyoto Encyclopedia of Genes and Genome (KEGG) database were used to perform functional enrichment analysis on selected genes and to explore the potential function and pathway of genes. Univariate Cox analysis, lasso regression analysis and multivariate Cox analysis were used to screen out genes related to ACC TME and to establish risk score (RS) model for ACC patients. The receiver operating characteristic (ROC) curve was used to evaluate the prognostic value of RS. The data sets GSE33371 and GSE19750 of Gene Expression Omnibus (GEO) were used as external validation sets to validate the prognostic model. The data of 79 ACC patients were extracted from the TCGA database, and the clinicopathological factors and the RS of the established prognostic model were included in the Cox regression analysis to obtain the prognostic factors of ACC patients.Results:According to the immune score and stromal score, 1 205 differentially expressed genes from intersection of both scores were screened out by using VennDiagram, including 833 up-regulated genes and 372 down-regulated genes. After continuing the regression analysis and screening of differentially expressed genes, the ACC prognostic model containing 9 TME-related genes (GREB1, POU4F1, HIC1, HOXC9, CACNB2, RAB27B, ZIC2, C3, CYP2D6) was finally constructed, that was, RS = GREB1×0.223 6+POU4F1×0.671 7+HIC1×0.167 5+HOXC9×0.211 3+CACNB2×0.156 0+RAB27B×0.956 5+ZIC2×0.582 7+C3×(-0.003 1)+CYP2D6×0.819 3. The area under the curve (AUC) of ROC for the 1, 3, and 5-year overall survival of 79 ACC patients predicted by the model in the TCGA database was 0.876, 0.919, 0.917, respectively. In the GEO validation set, the AUC of the 1, 3, and 5-year overall survival for 45 ACC patients predicted by the model was 0.689, 0.704, and 0.708, respectively, indicating that the model had a high prediction accuracy for survival results of ACC patients. Cox regression analysis on the data of 79 ACC patients in the TCGA database showed that the TME-related gene prognostic model RS was an independent factor influencing the prognosis of ACC patients ( HR = 1.011, 95% CI 1.005-1.016, P < 0.01). Conclusions:The established ACC TME-related gene prognostic model can be used to predict the prognosis of ACC patients. The model including 9 genes may become a new target for studying the pathogenesis and immunotherapy of ACC, and it is worthy of further research.

Analysis of Drug Use in a Lung Squamous Carcinoma Patient with Immune Checkpoint Inhibitors Associated Adrenocortical Insufficiency / 中国药房

OBJECTIVE：To explore the role of clinical pharmacists in the diagnosis and tre atment of immune checkpoint inhibitor related adrenocortical insufficiency ，so as to provide reference for the diagnosis and treatment of similar cases. METHODS：A lung squamous carcinoma patient with tumor immunotherapy ，who received long-term intravenous infusion of Pembrolizumab injection ，was hospitalized for more than 10 days due to fatigue and poor appetite. The clinical pharmacists consulted the literature and evaluated the correlation of ADR on the basis of patient ’s physical examination results （plasma cortisol 16.41 nmol/L at 8：00 in the morning on the second day of admission ，adrenocorticotropic hormone 8.39 pg/mL，serum electrolyte sodium 126.00 mmol/L，chlorine 88.00 mmol/L，etc.）and medication history （receiving the treatment with immune checkpoint inhibitors）. The clinical pharmacists assisted the doctor to confirm that the patient was adrenocortical insufficiency ，and the correlation with pembrolizumab was “very likely ”. On the basis of discontinuation of pembrolizumab ，it was recommended to take Hydrocortisone tablets orally （20 mg at 8：00 in the morning and 10 mg at 4：00 in the afternoon ）for glucocorticoid physiological dose substitution therapy. Before treatment ，the patient and his families should be educated on the use of drugs ，pharmaceutical care should be implemented to evaluate the glucocorticoid doseand efficacy during treatment ，and medication education was carried out at discharge. RESULTS ：The doctor adopted theli_xyan suggestions of the clinical pharmacist. On the 5th day after @126.com administration，the symptoms of adrenocortical insufficiency were significantly impro ved，and the patient was discharged with medicine. CONCLUSIONS ：During the treatment of the patient ， the clinical pharmacist gave full play to his pharmaceutical expertise ，assisted the doctor to formulate an appropriate medication plan，and carried out medication education for the patient and his family members ，so as to ensure the effectiveness and safety of the patient ’s medication. It is suggested that the baseline examination of patients should be improved before immune checkpoint inhibitor treatment ，including physical examination and plasma cortisol level at 8：00 in the morning ；the frequency of monitoring plasma cortisol level and serum electrolyte level at 8：00 in the morning. should be increased during medication ；the patients should be followed up for at least 1 year after withdrawal ，so as to early detect and treat immune checkpoint inhibitor related adrenocortical dysfunction.

Risk factors and predictive model of adrenocortical insufficiency in patients with traumatic brain injury / 世界急诊医学杂志（英文）

@#BACKGROUND: Neuroendocrine dysfunction after traumatic brain injury (TBI) has received increased attention due to its impact on the recovery of neural function. The purpose of this study is to investigate the incidence and risk factors of adrenocortical insufficiency (AI) after TBI to reveal independent predictors and build a prediction model of AI after TBI. METHODS: Enrolled patients were grouped into the AI and non-AI groups. Fourteen preset impact factors were recorded. Patients were regrouped according to each impact factor as a categorical variable. Univariate and multiple logistic regression analyses were performed to screen the related independent risk factors of AI after TBI and develop the predictive model. RESULTS: A total of 108 patients were recruited, of whom 34 (31.5%) patients had AI. Nine factors (age, Glasgow Coma Scale [GCS] score on admission, mean arterial pressure [MAP], urinary volume, serum sodium level, cerebral hernia, frontal lobe contusion, diffuse axonal injury [DAI], and skull base fracture) were probably related to AI after TBI. Three factors (urinary volume [X4], serum sodium level [X5], and DAI [X8]) were independent variables, based on which a prediction model was developed (logit P= -3.552+2.583X4+2.235X5+2.269X8). CONCLUSIONS: The incidence of AI after TBI is high. Factors such as age, GCS score, MAP, urinary volume, serum sodium level, cerebral hernia, frontal lobe contusion, DAI, and skull base fracture are probably related to AI after TBI. Urinary volume, serum sodium level, and DAI are the independent predictors of AI after TBI.

Síndrome de Cushing provocado por carcinoma suprarrenal gigante. Caso clínico

We report a previously healthy 34-year-old woman, presenting with a seven-month history of arterial hypertension, amenorrhea, weight gain, facial edema, acne, hirsutism and low back pain. A CT scan showed a right adrenal mass of 18 × 13 × 12.5 cm, and multiple vertebral and rib fractures. The hormonal study confirmed Cushing's Syndrome. Ketoconazole, spironolactone, cotrimoxazole, calcium / vitamin D were started. An adrenalectomy with a right nephrectomy were performed. The excised tumor measured 16 cm and weighed 1.55 kg. There was tumor embolism and a 4 mm soft tissue involvement (pT3NxMx). The right kidney was free of tumor. The patient was treated with chemotherapy (etoposide plus cisplatin). Study of vertebral fractures with magnetic resonance (MRI) showed crush fractures, without images of metastatic bone lesions. One year after surgery, a CT scan showed no signs of tumor recurrence. The patient was lost from follow-up thereafter.

Giant non-functioning adrenocortical carcinoma: an unusual tumour presentation

Adrenocortical carcinoma is a rare tumour with incidence of 1 per million all over the world. Age distribution is bimodal with peaks occurring at 5-20 years and 40-50 years. Tumours greater than 9 cm commonly involve inferior vena cava (IVC) or right side of heart. 80 percent of the carcinomas are functional. We describe a case report of a rare tumour of a non-functioning adrenocortical carcinoma in a 40 year old female having incidence of 0.5/million world over with no extra-adrenal spread at presentation without IVC or right heart involvement in spite of having a large size of 12 cm. Patient underwent successful surgical extirpation with adjuvant radiotherapy with regular follow ups and disease free survival since the surgery. It is imperative to determine hormone levels in symptomatic and asymptomatic patients with adrenal masses. The possibility of adrenocortical carcinoma should not be ruled out in a functioning/non-functioning tumour. All solid incidentalomas on computed tomography (CT)/magnetic resonance imaging (MRI) greater than 5 cm in size should be removed surgically with adjuvant therapy consideration after histopathological reporting.