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    Adrenal tumour bigger than 5 cm - what could it be? An analysis of 139 cases.
    Endokrynol Pol 2017 Jun 12. Epub 2017 Jun 12.
    Department of Surgery Clinic of Oncology Memorial M. Sklodowska-Curie Cancer Institute, Warsaw, Poland.
    Introduction: There is an increasing number of adrenal being tumours discovered incidentally during imaging examinations performed for many different indications. Radiological findings suggesting adrenal pathology may be caused by true adrenal tumours or by other retroperitoneal masses. Generally, the larger the tumour, the higher the possibility of adrenal cancer. Read More

    Macrolides selectively inhibit mutant KCNJ5 potassium channels that cause aldosterone-producing adenoma.
    J Clin Invest 2017 Jun 12. Epub 2017 Jun 12.
    Department of Genetics and Howard Hughes Medical Institute, Yale University School of Medicine, New Haven, Connecticut, USA.
    Aldosterone-producing adenomas (APAs) are benign tumors of the adrenal gland that constitutively produce the salt-retaining steroid hormone aldosterone and cause millions of cases of severe hypertension worldwide. Either of 2 somatic mutations in the potassium channel KCNJ5 (G151R and L168R, hereafter referred to as KCNJ5MUT) in adrenocortical cells account for half of APAs worldwide. These mutations alter channel selectivity to allow abnormal Na+ conductance, resulting in membrane depolarization, calcium influx, aldosterone production, and cell proliferation. Read More

    Higher Glucocorticoid Replacement Doses Are Associated with Increased Mortality in Patients with Pituitary Adenoma.
    Eur J Endocrinol 2017 Jun 8. Epub 2017 Jun 8.
    D Olsson, Department of Endocrinology, Sahlgrenska University Hospital, Göteborg, SE-413 45, Sweden
    Objective: Patients with secondary adrenal insufficiency (AI) have an excess mortality. The objective was to investigate the impact of the daily glucocorticoid replacement dose on mortality in patients with hypopituitarism due to non-functioning pituitary adenoma (NFPA).

    Method: Patients with NFPA were followed between years 1997- 2014 and cross-referenced with the National Swedish Death Register. Read More

    Glucose metabolism disorders in patients with non-functioning adrenal adenomas: single-centre experience.
    Endokrynol Pol 2017 Jun 6. Epub 2017 Jun 6.
    Department of Endocrinology and Diabetology, Nicolaus Copernicus University in Toruń, Collegium Medicum in Bydgoszcz ul. Skłodowskiej-Curie 9, 85-094 Bydgoszcz.
    Introduction: The presence of glucose metabolism disorders and their possible correlation with degree of cortisol secretion were evaluated in patients with non-functioning adrenal incidentalomas (NFAIs).

    Material And Methods: The study group consisted of 131 patients with hormonally inactive adrenal incidentalomas. In each patient, besides hormonal and radiological evaluation, was assessed for fasting glucose and insulin concentrations, and the oral glucose tolerance test (OGTT) was performed in all participants without previous history of glucose disturbances. Read More

    Central precocious puberty secondary to adrenocortical adenoma in a female child: case report and review of the literature.
    J Pediatr Adolesc Gynecol 2017 May 31. Epub 2017 May 31.
    Professor, Department of Pediatric Surgery, Celal Bayar University, School of Medicine, Manisa, TURKEY.
    Background: Pediatric adrenocortical tumors are rare but significant causes of virilization and peripheral precocious puberty (PPP).

    Case: A 4-year-old girl presented with development of breast, pubic hair, and facial acne. Her bone age was advanced, and gonadotropins did not elevate in gonadotropin-releasing hormone (GnRH) test. Read More

    Myxoid Adrenocortical Adenoma: A report of two cases and literature review.
    Pathol Res Pract 2017 Jul 20;213(7):857-859. Epub 2017 Apr 20.
    Department of Pathology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China. Electronic address:
    Myxoid adrenocortical adenomas are uncommon. There were only 61 cases reports documented, and the tumors are tended to be misdiagnosed in virtue of being rare and distinctive histological features. Recently we encountered two myxoid adrenocortical adenoma cases of a 31-year-old Chinese woman and a 45-year-old Chinese man. Read More

    Analysis of histological and immunohistochemical patterns of benign and malignant adrenocortical tumors by computerized morphometry.
    Pathol Res Pract 2017 Jul 9;213(7):815-823. Epub 2017 Mar 9.
    Department of Internal Medicine, Endocrinology Unit, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.
    Diagnosis of benign and purely localized malignant adrenocortical lesions is still a complex issue. Moreover, histology-based diagnosis may suffer of a moment of subjectivity due to inter- and intra-individual variations. The aim of the present study was to assess, by computerized morphometry, the morphological features in benign and malignant adrenocortical neoplasms. Read More

    A exceptional collision tumor of primary adrenal angiosarcoma and non-functioning adrenocortical adenoma.
    Pathol Res Pract 2017 Jun 19;213(6):702-705. Epub 2017 Apr 19.
    Department of Anatomic pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, 812-8582, Japan. Electronic address:
    Primary adrenal angiosarcoma is an extremely rare vascular tumor. We report a case of a 63-year-old man with a collision tumor of epithelioid angiosarcoma and adrenocortical adenoma of the right adrenal gland. The adrenal tumor was incidentally observed by a preoperative computed tomography (CT) scan of penis squamous cell carcinoma. Read More

    PRKAR1A mutation causing pituitary-dependent Cushing disease in a patient with Carney complex.
    Eur J Endocrinol 2017 May 18. Epub 2017 May 18.
    A Gessl, Department of Medicine III, Clinical Division of Endocrinology & Metabolism, Medical University of Vienna, Vienna, Austria
    Context: Carney complex (CNC) is an autosomal dominant condition caused, in most cases, by an inactivating mutation of the PRKAR1A gene, which encodes for the type 1 alpha regulatory subunit of protein kinase A. CNC is characterized by the occurrence of endocrine overactivity, myxomas and typical skin manifestations. Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine disease observed in CNC. Read More

    Reninoma coexisting with adrenal adenoma during pregnancy: A case report.
    Oncol Lett 2017 May 3;13(5):3186-3190. Epub 2017 Mar 3.
    Department of Diagnostic Radiology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China.
    Reninoma is a rare form of secondary hypertension. The present study reported a case of reninoma that coexisted with an adrenal adenoma in a young female with secondary hypertension during pregnancy. The patient, a 31-year-old female exhibiting hypertension for >1 year, developed a mass in the right adrenal gland, which was detected by an ultrasound scan in the 33rd gestational week. Read More

    Expression and Histopathological Significance of Disabled-2 in Aldosterone-Producing Adenoma.
    Horm Metab Res 2017 May 17. Epub 2017 May 17.
    Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, P. R. China.
    The current pathological diagnosis of aldosterone-producing adenoma (APA) is challenging because no histological markers of aldosterone production are available in routine practice. A previous study demonstrated that Disabled-2 (DAB2) is a specific marker of the zona glomerulosa (ZG) in rodents. The aim of the present study was to investigate the significance of immunohistochemical staining to detect DAB2 in the adrenal tissue of patients with APA. Read More

    Laparoscopic Adrenalectomy for Conn's Syndrome is Beneficial to Patients and is Cost Effective in England.
    J Invest Surg 2017 May 12:1-7. Epub 2017 May 12.
    a Department of Endocrine Surgery, Churchill Hospital , Oxford University Hospitals NHS Trust , Oxford , United Kingdom.
    Purpose: Adrenalectomy has the potential to cure or improve the control of hypertension in patients with primary hyperaldosteronism due to unilateral adrenal adenoma (Conn's syndrome). This study assesses the patients' perception of, and costs associated with, laparoscopic adrenalectomy for Conn's syndrome.

    Materials And Methods: Clinical, radiological, operative, and pathological data were collected on patients undergoing adrenalectomy for Conn's syndrome over 8-years period in a UK tertiary referral center. Read More

    Robot-assisted adrenalectomy: indications and drawbacks.
    Updates Surg 2017 May 12. Epub 2017 May 12.
    Department of Digestive, Hepatobiliary, Endocrine, and Oncology Surgery, University of Lorraine, CHU Nancy-Brabois (Adultes), 54511, Vandoeuvre-Les-Nancy, France.
    Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers. Read More

    A case of confirmed primary hyperaldosteronism diagnosed despite normal screening investigations.
    N Z Med J 2017 May 12;130(1455):129-132. Epub 2017 May 12.
    Department of Surgery, Wellington Regional Hospital, Newtown, Wellington.
    Primary hyperaldosteronism is a common cause of hypertension in the adult population. We report a case of histologically and biochemically confirmed hyperaldosteronism related to an adrenal adenoma, where initial screening and biochemical tests were potentially misleading. The case highlights the importance of clinical suspicion in the current diagnostic approach to primary hyperaldosteronism. Read More

    Portal Vein Thrombosis in the Setting of Newly Diagnosed Cushing's Syndrome.
    J Investig Med High Impact Case Rep 2017 Apr-Jun;5(2):2324709617703672. Epub 2017 Apr 10.
    Southern Illinois University, Springfield, IL, USA.
    The hallmark manifestations of Cushing's syndrome (CS) are well known, but hypercoagulability is perhaps least recognized. Patients with CS are at increased risk of both spontaneous and postoperative thromboembolism, with the significant majority of events occurring in the lower extremity and pulmonary venous circulations. We present a case of portal vein thrombosis (PVT) occurring in the setting of newly diagnosed CS due to a left adrenal adenoma. Read More

    [Laparoscopic adrenalectomy: advantages of the minimally invasive approach].
    Cir Pediatr 2016 Oct 10;29(4):162-165. Epub 2016 Oct 10.
    Hospital Universitari i Politécnic La Fe. Valencia.
    Aim: To describe our experience as a tertiary center on the use of laparoscopic adrenal surgery in children.

    Material And Methods: A descriptive, retrospective study of patients with pathologic adrenal masses undergoing laparoscopic adrenal surgery, between 2012 and 2015. Epidemiological variables, surgical technique, complications and follow-up were studied. Read More

    Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
    Endocr Pathol 2017 May 6. Epub 2017 May 6.
    Cancer Molecular Pathology, School of Medicine and Menzies Health Institute Queensland, Griffith University, Gold Coast, Q4222, Australia.
    The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours. Read More

    Cholesterol delivery to the adrenal glands estimated by adrenal venous sampling: An in vivo model to determine the contribution of circulating lipoproteins to steroidogenesis in humans.
    J Clin Lipidol 2017 May - Jun;11(3):733-738. Epub 2017 Apr 5.
    Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
    Background: Cholesterol, required for adrenal steroid hormone synthesis, is at least in part derived from circulating lipoproteins. The contribution of high-density lipoproteins (HDL) and low-density lipoproteins (LDL) to adrenal steroidogenesis in humans is unclear.

    Objective: The aim of the study was to determine the extent to which HDL and LDL are taken up by the adrenal glands using samples obtained during adrenal venous sampling (AVS). Read More

    Circadian Plasma Cortisol Measurements Reflect Severity of Hypercortisolemia in Children with Different Etiologies of Endogenous Cushing Syndrome.
    Horm Res Paediatr 2017 Apr 21. Epub 2017 Apr 21.
    Background: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet.

    Methods: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6). Circadian plasma cortisol measurements were obtained at 11: 30 p. Read More

    Steroid metabolome analysis reveals prevalent glucocorticoid excess in primary aldosteronism.
    JCI Insight 2017 Apr 20;2(8). Epub 2017 Apr 20.
    Background: Adrenal aldosterone excess is the most common cause of secondary hypertension and is associated with increased cardiovascular morbidity. However, adverse metabolic risk in primary aldosteronism extends beyond hypertension, with increased rates of insulin resistance, type 2 diabetes, and osteoporosis, which cannot be easily explained by aldosterone excess.

    Methods: We performed mass spectrometry-based analysis of a 24-hour urine steroid metabolome in 174 newly diagnosed patients with primary aldosteronism (103 unilateral adenomas, 71 bilateral adrenal hyperplasias) in comparison to 162 healthy controls, 56 patients with endocrine inactive adrenal adenoma, 104 patients with mild subclinical, and 47 with clinically overt adrenal cortisol excess. Read More

    Case report: ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing's disease.
    Diagn Pathol 2017 Apr 18;12(1):34. Epub 2017 Apr 18.
    Department of Pathology, The Ohio State University Wexner Medical Center, 410 W 10th Avenue, Columbus, OH, 43210, USA.
    Background: Pituitary carcinoma is a rare entity requiring the presence of metastasis to confirm its malignant potential. We report a case of pituitary carcinoma and discuss the diagnosis and management of this lesion in relation to the existing literature.

    Case Presentation: The patient is a 51-year-old woman with Cushing's disease and intact adrenal glands who was diagnosed with metastatic pituitary carcinoma to the liver, 29 months after initial resection of an ACTH-secreting primary atypical pituitary adenoma (APA). Read More

    Subtype Diagnosis of Primary Aldosteronism: Is Adrenal Vein Sampling Always Necessary?
    Int J Mol Sci 2017 Apr 17;18(4). Epub 2017 Apr 17.
    Division of Internal Medicine and Hypertension Unit, Department of Medical Sciences, University of Torino, Via Genova 3, 10126 Torino, Italy.
    Aldosterone producing adenoma and bilateral adrenal hyperplasia are the two most common subtypes of primary aldosteronism (PA) that require targeted and distinct therapeutic approaches: unilateral adrenalectomy or lifelong medical therapy with mineralocorticoid receptor antagonists. According to the 2016 Endocrine Society Guideline, adrenal venous sampling (AVS) is the gold standard test to distinguish between unilateral and bilateral aldosterone overproduction and therefore, to safely refer patients with PA to surgery. Despite significant advances in the optimization of the AVS procedure and the interpretation of hormonal data, a standardized protocol across centers is still lacking. Read More

    Physiological and Pathological Roles in Human Adrenal of the Glomeruli-Defining Matrix Protein NPNT (Nephronectin).
    Hypertension 2017 Jun 17;69(6):1207-1216. Epub 2017 Apr 17.
    From the Clinical Pharmacology Unit, Centre for Clinical Investigation, Addenbrooke's Hospital (A.E.D.T., S.G., J.Z., M.J.B.), Tissue Bank, Department of Histopathology, Addenbrooke's Hospital (W.Z.), NIHR Cambridge Biomedical Research Centre, Addenbrooke's Hospital (M.G.), MRC Cancer Unit, Hutchison/MRC Research Centre (T.I.J.), and Metabolic Research Laboratories, Wellcome Trust-MRC Institute of Metabolic Science (M.G.), University of Cambridge, United Kingdom; Centre for Clinical Pharmacology, William Harvey Research Institute, Barts and the London School of Medicine & Dentistry, Queen Mary University of London, United Kingdom (J.Z., M.J.B.); Division of Endocrinology, Department of Medicine, The University of Mississippi Medical Centre, Jackson (C.E.G.-S.); and Research and Medicine Services, G.V. (Sonny) Montgomery VA Medical Centre, Jackson, MS (C.E.G.-S.).
    Primary aldosteronism is a common cause of hypertension, which becomes refractory if undiagnosed, but potentially curable when caused by an aldosterone-producing adenoma (APA). The discovery of somatic mutations and differences in clinical presentations led to recognition of small but common zona glomerulosa (ZG)-like adenomas, distinct from classical large zona fasciculata-like adenomas. The inverse correlation between APA size and aldosterone synthase expression prompted us to undertake a systematic study of genotype-phenotype relationships. Read More

    Posterior retroperitonoscopic adrenalectomy; a back door access with an unusually rapid learning curve.
    Updates Surg 2017 Apr 12. Epub 2017 Apr 12.
    Division of Endocrine Surgery, Department of Surgical Pathology, University Hospital of Pisa, Via Paradisa 2, 56124, Pisa, Italy.
    Posterior retroperitonoscopic adrenalectomy (PRA) has become a standard approach to the adrenal gland. The aim of this study was to report an initial experience with the procedure following a proper preparatory phase highlighting the rapidity, safety and effectiveness by which it could be introduced into a surgeon's practice. Between May 2015 and July 2016, 14 PRAs were performed in 14 patients (9 females and 5 males). Read More

    Histopathological and genetic characterization of aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion: a case series.
    Endocrine 2017 Apr 12. Epub 2017 Apr 12.
    Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität, Munich, Germany.
    Purpose: Aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion are reported in an increasing number of patients. Five aldosterone-producing adenomas from patients with primary aldosteronism and subclinical hypercortisolism were examined.

    The Aims Of Our Study Were: (1) to analyze pathological features and immunohistochemical expression of CYP11B1 (11β-hydroxylase) and CYP11B2 (aldosterone synthase) in these tumors; (2) to investigate somatic mutations involved in adrenal steroid hypersecretion and/or tumor growth. Read More

    Can Imaging Predict Subclinical Cortisol Secretion in Patients With Adrenal Adenomas? A CT Predictive Score.
    AJR Am J Roentgenol 2017 Jul 12;209(1):122-129. Epub 2017 Apr 12.
    2 Endocrinology Unit, Department of Medical and Surgical Sciences, Alma Mater Studiorum-University of Bologna, S. Orsola-Malpighi Hospital, Bologna, Italy.
    Objective: The aim of this study is to determine whether any correlation between CT findings and functional parameters exists to predict subclinical glucocorticoid secretion.

    Materials And Methods: This is a retrospective database study of 55 patients with incidentally discovered adenomas, investigated through CT with an adrenal protocol, assessing diameters and attenuation values on the unenhanced and contrast-enhanced phases. Patients underwent blood cortisol and corticotropin evaluation and overnight dexamethasone suppression test (DST), in accordance with clinical recommendations. Read More

    Somatic and inherited mutations in primary aldosteronism.
    J Mol Endocrinol 2017 Jul 11;59(1):R47-R63. Epub 2017 Apr 11.
    INSERMUMRS_970, Paris Cardiovascular Research Center, Paris, France.
    Primary aldosteronism (PA), the most common form of secondary hypertension, is caused in the majority of cases by unilateral aldosterone-producing adenoma (APA) or bilateral adrenal hyperplasia. Over the past few years, somatic mutations in KCNJ5, CACNA1D, ATP1A1 and ATP2B3 have been proven to be associated with APA development, representing more than 50% of sporadic APA. The identification of these mutations has allowed the development of a model for APA involving modification on the intracellular ionic equilibrium and regulation of cell membrane potential, leading to autonomous aldosterone overproduction. Read More

    Primary pigmented nodular adrenocortical disease: literature review and case report of a 6-year-old boy.
    J Pediatr Endocrinol Metab 2017 May;30(5):603-609
    University of Novi Sad, Faculty of Medicine, Novi Sad.
    Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. Read More

    Prevalence and Clinical Manifestations of Primary Aldosteronism Encountered in Primary Care Practice.
    J Am Coll Cardiol 2017 Apr;69(14):1811-1820
    Division of Internal Medicine and Hypertension Unit, Department of Medical Sciences, University of Torino, Torino, Italy. Electronic address:
    Background: Despite being widely recognized as the most common form of secondary hypertension, among the general hypertensive population the true prevalence of primary aldosteronism (PA) and its main subtypes, aldosterone-producing adenoma (APA) and bilateral adrenal hyperplasia (BAH), remains a matter of debate.

    Objectives: This study sought to determine the prevalence and clinical phenotype of PA in a large cohort of unselected patients with hypertension, consecutively referred to our hypertension unit, by 19 general practitioners from Torino, Italy.

    Methods: Following withdrawal from all interfering medications, patients were screened for PA using the ratio of serum aldosterone to plasma renin activity. Read More

    Adrenal Vein Sampling for Primary Aldosteronism: A 2-Week Protocol for Withdrawal of Renin-Stimulating Antihypertensives.
    Cardiovasc Intervent Radiol 2017 Apr 4. Epub 2017 Apr 4.
    Department of Radiology, Hospital of the University of Pennsylvania, 1 Silverstein, 3400 Spruce St, Philadelphia, PA, 19104, USA.
    Objective: For evaluation of primary aldosteronism, international guidelines recommend a 4-6 week withdrawal of spironolactone, eplerenone, and amiloride prior to adrenal vein sampling (AVS). It is not always feasible to withdraw these drugs in patients with severe hypertension and hypokalemia. We present our experience evaluating the efficacy and clinical outcomes of a 2-week protocol for withdrawal of renin-stimulating antihypertensives prior to AVS. Read More

    Microinsertions in PRKACA cause activation of the protein kinase A pathway in cardiac myxoma.
    J Pathol 2017 Jun;242(2):134-139
    Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan.
    Cardiac myxoma is the most common cardiac tumour. Most lesions occur sporadically, but occasional lesions develop in patients with Carney complex, a syndrome characterized by cardiac myxoma, spotty pigmentation, and endocrine overactivity. Two-thirds of patients with Carney complex harbour germline mutations in PRKAR1A, which encodes the type I regulatory subunit of protein kinase A (PKA). Read More

    Genetic and Histopathologic Intertumor Heterogeneity in Primary Aldosteronism.
    J Clin Endocrinol Metab 2017 Jun;102(6):1792-1796
    Center for Adrenal Disorders, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115.
    Context: Whether primary aldosteronism (PA) is the consequence of a monoclonal or multiclonal process is unclear.

    Case Description: A 48-year-old man with severe bilateral PA refractory to medical therapy underwent unilateral adrenalectomy of the dominant adrenal. Although computed tomography showed three left-sided cortical nodules, postsurgical histopathology and genetic analysis revealed five different adrenocortical adenomas. Read More

    Spinal adrenal cortical adenoma associated with Beckwith-Wiedemann syndrome: case report and review of the literature.
    Childs Nerv Syst 2017 Jun 1;33(6):1009-1013. Epub 2017 Apr 1.
    Department of Pathology, University Hospital La Paz, Madrid, Spain.
    Purpose: Ectopic adrenal cortical adenoma in the spinal region is extremely rare. The majority of cases of ectopic adrenocortical tissue are found along the path of embryonic migration within the urogenital tract. Beckwith-Wiedemann syndrome (BWS) is a pediatric overgrowth disorder involving a predisposition to tumor development, including adrenal lesions. Read More

    Intrafamilial Phenotypic Variability and Consequences of Non-Compliance with Treatment in Congenital Adrenal Hyperplasia and Congenital Hypothyroidism within a Single Family
    Horm Res Paediatr 2017 Mar 30. Epub 2017 Mar 30.
    Background: Coexistence of congenital adrenal hyperplasia (CAH) and congenital hypothyroidism (CH) due to TG mutation in the same non-consanguineous family is rare.

    Case Series: We report 4 siblings born to unrelated parents, the father being an asymptomatic carrier of homozygous p.V281L and heterozygous p. Read More

    Differentiating pheochromocytoma from lipid-poor adrenocortical adenoma by CT texture analysis: feasibility study.
    Abdom Radiol (NY) 2017 Mar 29. Epub 2017 Mar 29.
    Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Shuaifuyuan No. 1, Wangfujing Street, Dongcheng District, Beijing, 100730, China.
    Objectives: To investigate the feasibility of using CT texture analysis (CTTA) to differentiate pheochromocytoma from lipid-poor adrenocortical adenoma (lp-ACA).

    Methods: Ninety-eight pheochromocytomas and 66 lp-ACAs were included in this retrospective study. CTTA was performed on unenhanced and enhanced images. Read More

    [A clinical analysis of 123 cases of primary empty sella].
    Zhonghua Nei Ke Za Zhi 2017 Apr;56(4):268-272
    Department of Endocrinology and Metabolism, General Hospital of Tianjin Medical University, Tianjin 300052, China.
    Objective: This study was conducted to analyze the clinical characteristics and pituitary function of patients with primary empty sella (PES). Methods: The clinical data from 123 hospitalized adult patients with PES from January 2010 to May 2016 were retrospectively studied. Results: (1) The average age of the 123 (male 43, female 80) PES patients was (59. Read More

    From benign adrenal incidentaloma to adrenocortical carcinoma: an exceptional random event.
    Eur J Endocrinol 2017 Jun 27;176(6):K15-K19. Epub 2017 Mar 27.
    Department of EndocrinologyCenter for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France
    New European guidelines for the management of adrenal incidentalomas were recently released. One of the most novel recommendations is to stop following patients when they present a typical, small and non-secreting adenoma. We report here the case of a 71-year-old man with such an adenoma, who developed an adrenocortical carcinoma (ACC) fourteen years later, with subsequent metastases and death. Read More

    Endocr Pract 2017 Jun 23;23(6):680-689. Epub 2017 Mar 23.
    Objective: With the increased emphasis on personalized and individualized medicine, the American Association of Clinical Endocrinologists Adrenal Scientific Committee has developed a series of articles to update members on personalized medicine as it applies to adrenal diseases.

    Methods: We synthesized literature reviews, guidelines from professional societies, and personal experience.

    Results: Since Conn described primary aldosteronism (PA) over 60 years ago, debate has raged about the prevalence of PA in the hypertensive population, the wisdom of broadly screening for PA, and prudent approaches to evaluate and manage these patients. Read More

    Endocr Pract 2017 Jun 23;23(6):672-679. Epub 2017 Mar 23.
    Objective: Discuss exciting new research in the area of adrenal disorders that has emerged in the last few years. Advances in genetics, biochemical diagnosis, and imaging modalities that have set new standards for diagnosis and treatment are described.

    Methods: A literature review was conducted on adrenal disorders using PubMed. Read More

    Evidence for persistent organochlorine pollutants in the human adrenal cortex.
    J Appl Toxicol 2017 Mar 23. Epub 2017 Mar 23.
    Institute of Clinical Physiology, National Research Council (CNR), Pisa, Italy.
    Environmental pollutants may act as endocrine disruptors in animals. Organochlorine pesticides (OCPs) and polychlorinated biphenyls (PCBs) enter the food chain and may accumulate in the fatty animal tissues, including adrenals. To our knowledge, no previous study has investigated their presence in the human normal adrenal (NA) cortex and aldosterone-producing adenomas (APA). Read More

    A Case of Malignant Pheochromocytoma Presenting 7 Years After the Initial Surgery.
    Ochsner J 2017 ;17(1):128-131
    The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA ; Department of Surgery, Ochsner Clinic Foundation, New Orleans, LA.
    Background: Pheochromocytoma (PHEO) is a rare tumor of the adrenal medulla and sympathetic ganglion that produces the catecholamines norepinephrine and epinephrine. Traditionally, approximately 10% of PHEOs were thought to be malignant, but recent developments in PHEO research have noted that specific genetic mutations are associated with higher risk of metastatic spread.

    Case Report: We report the case of a 71-year-old female who presented with abdominal pain in September 2009 when she was 64 years old. Read More

    Neuroendocrine Tumors in the Stomach, Duodenum, and Pancreas Accompanied by Novel MEN1 Gene Mutation.
    Korean J Gastroenterol 2017 Mar;69(3):181-186
    Division of Gastroenterology, Department of Internal Medicine, Presbyterian Medical Center, Jeonju, Korea.
    Multiple endocrine neoplasia type 1 (MEN1) syndrome is a relatively rare disease, characterized by the occurrence of multiple endocrine tumors in the parathyroid and pituitary glands as well as the pancreas. Here, we report a case of MEN1 with neuroendocrine tumors (NETs) in the stomach, duodenum, and pancreas. A 53-year-old man visited our hospital to manage gastric NET. Read More

    Modified approach to the characterization of adrenal nodules using a standard abdominal magnetic resonance imaging protocol.
    Radiol Bras 2017 Jan-Feb;50(1):19-25
    Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA, and Hospital Garcia de Orta, Almada, Portugal.
    Objective: To describe a modified approach to the evaluation of adrenal nodules using a standard abdominal magnetic resonance imaging protocol.

    Materials And Methods: Our sample comprised 149 subjects (collectively presenting with 132 adenomas and 40 nonadenomas). The adrenal signal intensity index was calculated. Read More

    Simultaneous assay of cortisol and dexamethasone improved diagnostic accuracy of the dexamethasone suppression test.
    Eur J Endocrinol 2017 Jun 15;176(6):705-713. Epub 2017 Mar 15.
    Department of Clinical ScienceUniversity of Bergen, Bergen, Norway.
    Objectives: The overnight dexamethasone (DXM) suppression test (DST) has high sensitivity, but moderate specificity, for diagnosing hypercortisolism. We have evaluated if simultaneous measurement of S-DXM may correct for variable DXM bioavailability and increase the diagnostic performance of DST, and if saliva (sa) is a feasible adjunct or alternative to serum.

    Design And Methods: Prospective study of DST was carried out in patients with suspected Cushing's syndrome (CS) (n = 49), incidentaloma (n = 152) and healthy controls (n = 101). Read More

    Diagnostic accuracy of virtual non-contrast enhanced dual-energy CT for diagnosis of adrenal adenoma: A systematic review and meta-analysis.
    Eur Radiol 2017 Mar 13. Epub 2017 Mar 13.
    Clinical Epidemiology Program, Ottawa Hospital Research Institute, University of Ottawa Department of Radiology, Room c159 Ottawa Hospital Civic Campus, 1053 Carling Ave., Ottawa, ON, K1Y 4E9, USA.
    Objective: To compare the diagnostic accuracy of dual-energy (DE) virtual non-contrast computed tomography (vNCT) to non-contrast CT (NCT) for the diagnosis of adrenal adenomas.

    Methods: Search of multiple databases and grey literature was performed. Two reviewers independently applied inclusion criteria and extracted data. Read More

    Efficacy of endoscopic endonasal transsphenoidal surgery for Cushing's disease in 230 patients with positive and negative MRI.
    Acta Neurochir (Wien) 2017 Jul 9;159(7):1227-1236. Epub 2017 Mar 9.
    Department of Neurosurgery, Hôpital Foch, Suresnes, France.
    Object: The primary objective was to assess the remission rate, and the secondary objectives were to evaluate the early complications and recurrence rate and to define the predictive factors for the remission and recurrence rates.

    Patients And Methods: This prospective single-center study included 230 consecutive patients, operated on by a single surgeon for Cushing's disease via a transsphenoidal endoscopic endonasal approach, over a 6-year period (2008-2013). The patients included in this series were all adults (>18 years of age), who presented with clinical and biological characteristics of Cushing's disease confirmed based on dedicated MRI pituitary imaging. Read More

    A successful case of pregnancy in a woman with ACTH-independent Cushing's syndrome treated with ketoconazole and metyrapone.
    Gynecol Endocrinol 2017 May 26;33(5):349-352. Epub 2017 Feb 26.
    b Department of Clinical Endocrinology and Metabolic Disease , Polish Mother's Memorial Hospital Research Institute , Lodz , Poland.
    Cushing's syndrome (CS) is a rare disease caused by a chronic excess of cortisol. Hypercortisolaemia may affect reproductive system leading to infertility in women. However, some of the patients remain fertile, although pregnancy is uncommon. Read More

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