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    High 18Fluor-DCFPyL Uptake in Adrenal Adenomas.
    Clin Nucl Med 2017 Aug 19. Epub 2017 Aug 19.
    From the *Department of Nuclear Medicine and Radiology, Noordwest Ziekenhuisgroep locatie Alkmaar, Alkmaar; and †Department of Nuclear Medicine, Zaans Medisch Centrum, Zaandam, the Netherlands.
    Radioisotope-labeled prostate-specific membrane antigen (PSMA) tracers have been proven accurate for detection of prostate cancer localizations. Uptake of those tracers in other malignant and benign lesions has been reported, including faint accumulation of Ga-PSMA-HBED-CC in adrenal adenoma. A 77-year-old man with prostate carcinoma was scanned with F-DCFPyL, a promising F-labeled PSMA ligand, for prostate-specific antigen progression while on luteinizing hormone-releasing hormone agonist therapy. Read More

    Adrenal Cushing's syndrome during pregnancy.
    Eur J Endocrinol 2017 Aug 17. Epub 2017 Aug 17.
    R Feelders, Internal Medicine, Erasmus MC, Rotterdam, Netherlands.
    Cushing syndrome (CS) during pregnancy is a rare condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of overlapping features like fatigue, weight gain, striae and emotional changes that can occur during normal pregnancy. Changes in maternal hormones and their binding proteins complicate assessment of glucocorticoid hormone levels during gestation. Read More

    High-Resolution, Accurate-Mass (HRAM) Mass Spectrometry Urine Steroid Profiling in the Diagnosis of Adrenal Disorders.
    Clin Chem 2017 Aug 16. Epub 2017 Aug 16.
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
    Background: Steroid profiling is a promising diagnostic tool for those with adrenal tumors, Cushing syndrome (CS), and disorders of steroidogenesis. Our objective was to develop a multiple-steroid assay using liquid-chromatography, high-resolution, accurate-mass mass spectrometry (HRAM LC-MS) and to validate the assay in patients with various adrenal disorders.

    Methods: We collected 24-h urine samples from 114 controls and 71 patients with adrenal diseases. Read More

    [Fortuitous discovery of an adrenal mass following spontaneous retroperitoneal hemorrhage].
    Rev Med Liege 2017 Jul;72(7-8):358-362
    Service de Chirurgie Digestive, CH Bois de l'Abbaye, Seraing, Belgique.
    We report a case of spontaneous retroperitoneal haemorrhage by bleeding of an adrenal adenoma. Retroperitoneal haemorrhages are rarely encountered in practice, but must be rapidly diagnosed due to the life threatening risk. The etiologies, clinical assessment, management and differential diagnosis of spontaneous retroperitoneal haemorrhages are discussed. Read More

    Case Report: An incidentaloma that catches your eye - adrenal myelolipoma.
    F1000Res 2017 18;6:1140. Epub 2017 Jul 18.
    WONCA Polaris - USA, Bangkok, 10500, Thailand.
    Background: Adrenal incidentaloma refers to the incidental finding of a tumor in the adrenal gland, where nonfunctional forms are the most common variant. Myelolipoma is a rare (0.08-0. Read More

    Discordance between Imaging and Immunohistochemistry in Unilateral Primary Aldosteronism.
    Clin Endocrinol (Oxf) 2017 Aug 8. Epub 2017 Aug 8.
    Division of Metabolism, Endocrinology, and Diabetes, University of Michigan, Ann Arbor.
    Objective: Correct subtyping of primary aldosteronism (PA) is essential for good surgical outcomes. Adrenal vein sampling (AVS) and/or computed tomography (CT) are used for PA sub-classification. Clinical and/or biochemical improvement after surgery, however, is not always achieved in patients with presumed unilateral PA. Read More

    Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review.
    Diagn Pathol 2017 Aug 2;12(1):57. Epub 2017 Aug 2.
    Department of Pathology, Tokyo Metropolitan Geriatric Hospital, 35-2 Sakae-cho, Itabashi-ku, Tokyo, 173-0015, Japan.
    Background: Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown. Read More

    Pheochromocytoma with Synchronous Ipsilateral Adrenal Cortical Adenoma.
    World J Surg 2017 Jul 31. Epub 2017 Jul 31.
    Division of Subspecialty General Surgery, Mayo Clinic College of Medicine, 200 1st Street SW, Rochester, MN, 55901, USA.
    Background: Pheochromocytoma with synchronous ipsilateral adrenal cortical adenoma (PSCA) may present with mixed clinical, biochemical, and radiological features characteristic to each neoplasm subtype.

    Methods: All patients with a pathological diagnosis of pheochromocytoma were evaluated for an ipsilateral cortical adenoma from 1994 through 2015. Retrospectively extracted data included indications for adrenalectomy, diagnostic workup (biochemical and radiographic), operative characteristics, pathological findings, and postoperative complications. Read More

    Utility of MRI to Differentiate Clear Cell Renal Cell Carcinoma Adrenal Metastases From Adrenal Adenomas.
    AJR Am J Roentgenol 2017 Jul 25:W1-W8. Epub 2017 Jul 25.
    3 Department of Radiology, The Hospital of The University of Pennsylvania, The University of Pennsylvania, Philadelphia, PA.
    Objective: The purpose of this study is to compare MRI features of clear cell renal cell carcinoma (RCC) adrenal metastases and adenomas.

    Materials And Methods: Fifteen clear cell RCC adrenal metastases imaged with MRI were compared to 29 consecutive adenomas between 2006 and 2015. Two blinded radiologists assessed homogeneity (homogeneous vs heterogeneous), signal intensity (SI) decrease on chemical-shift MRI, and T2-weighted SI (isointense, mildly hyperintense, or markedly hyperintense) relative to muscle. Read More

    Primary bilateral adrenal nodular disease with Cushing's syndrome: varying aetiology.
    BMJ Case Rep 2017 Jul 24;2017. Epub 2017 Jul 24.
    Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Primary adrenal disorders contribute 20%â€"30% of patients with endogenous Cushing's syndrome. Most of the primary adrenal diseases are unilateral and include adenoma and adrenocortical carcinoma, whereas bilateral adrenal lesions are uncommon and include primary pigmented nodular adrenocortical disease, primary bilateral macronodular adrenocortical hyperplasia, isolated micronodular adrenocortical disease, bilateral adenomas or carcinomas, and rarely pituitary adrenocorticotropic hormone-dependent adrenal nodular disease. Cyclic adenosine monophosphate-dependent protein kinase A signalling is the major activator of cortisol secretion in primary adrenal nodular disorders. Read More

    Identifying unilateral disease in Chinese patients with primary aldosteronism by using a modified prediction score.
    J Hypertens 2017 Jul 20. Epub 2017 Jul 20.
    aDepartment of Hypertension bShanghai Institute of Hypertension, Shanghai Key Laboratory of Hypertension, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai cDepartment of Cardiology, Tongji Hospital, Tongji University dDepartment of Radiology eDepartment of Urology of Luwan Branch fDepartment of Urology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
    Objective: The current study aimed to evaluate the role of Küpers' score in predicting unilateral aldosteronism, and develop a modified score in Chinese patients with primary aldosteronism.

    Methods: The current retrospective study included 406 patients with primary aldosteronism who underwent successful adrenal venous sampling (AVS) and were divided into the unilateral (n = 211) and bilateral (n = 195) groups according to the AVS results. Normokalemia was noted in both the unilateral (n = 64) and bilateral groups (n = 84) when plasma and urinary aldosterone were measured. Read More

    Nationwide review of hormonally active adrenal tumors highlights high morbidity in pheochromocytoma.
    J Surg Res 2017 Jul 20;215:204-210. Epub 2017 Apr 20.
    Division of Endocrine Surgery, DeWitt Daughtry Family Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida.
    Background: Adrenal adenomas are benign tumors often discovered incidentally, and >70% are hormonally inactive. The remaining subset may produce excess aldosterone, cortisol, or catecholamine. Perioperative outcomes after adrenalectomy for such "hormonally active" tumors remain unclear. Read More

    Adrenalectomy for incidentaloma: lessons learned from a single-centre series of 274 patients.
    ANZ J Surg 2017 Jul 7. Epub 2017 Jul 7.
    Department of Digestive, Hepatobiliary and Endocrine Surgery, Referral Center for Rare Adrenal Diseases, Cochin Hospital, APHP, Paris, France.
    Background: Adrenal incidentalomas are increasingly diagnosed and include a wide spectrum of lesions from benign adenomas to secreting or malignant lesions. The aim of the present study is to report a large single-institution experience of patients undergoing surgery for adrenal incidentaloma with particular attention to their diagnosis and post-operative course and the evolution of surgical practice over time.

    Methods: From 1993 to 2013, 274 patients underwent adrenalectomy for incidentaloma. Read More

    A step by step approach in differential diagnosing of adrenal incidentaloma (epinephroma), (with comments on the new Clinical Practice Guidelines of the European Society of Endocrinology).
    Rom J Intern Med 2017 Jun 23. Epub 2017 Jun 23.
    .
    Objectives: To present a step by step approach for the diagnosis of adrenal incidentaloma (AI).

    Method: An extensive review of the literature was conducted, searching the Pub-Med and Google Scholar using the Mesh terms; Adrenal; Incidentaloma; Adrenal tumours; Radiology; Diagnosis. We also did a cross-referencing search of the literature. Read More

    A polymorphism in the CYP17A1 gene influences the therapeutic response to steroidogenesis inhibitors in Cushing's syndrome.
    Clin Endocrinol (Oxf) 2017 Jun 30. Epub 2017 Jun 30.
    Endocrinology/Medicine Department, Hospital Sant Pau and IIB, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII and Universitat Autònoma de Barcelona (UAB), Barcelona, Spain.
    Context: Steroidogenesis inhibitors, such as ketoconazole (KTZ) and metyrapone (MTP), are used to lower hypercortisolism in patients with Cushing's syndrome (CS). Cortisol normalization is not reached in all patients taking these medications.

    Objective: To test the hypothesis that variants in genes affecting steroidogenesis contribute to different responses to KTZ and/or MTP in patients with CS. Read More

    Diagnostic pitfalls of adrenal incidentaloma.
    Endokrynol Pol 2017 ;68(3):360-377
    Department of Endocrinology, Metabolism, and Internal Medicine, University of Medical Sciences, Poznan, Poland, Poland.
    Adrenal incidentaloma (AI) includes all lesions with diameter ≥ 1 cm found incidentally during imaging examinations not associated with suspected adrenal pathology. Every adrenal incidentaloma requires a detailed hormonal and imaging assessment. Initial diagnosis includes radiological phenotypical evaluation (particularly in CT, or in case of contraindications - in MRI) and biochemical assessment of tumour hormonal activity (which includes cortisol circadian rhythm, salivary cortisol concentration, 24-hour urinary free-cortisol and metanephrines test, short cortisol and dexamethasone suppression test, ACTH level assessment, aldosterone and ARO concentration, adrenal androgen concentration). Read More

    New evidences on the regulation of SF-1 expression by POD1/TCF21 in adrenocortical tumor cells.
    Clinics (Sao Paulo) 2017 Jun;72(6):391-394
    Departamento de Anatomia, Instituto de Ciencias Biomedicas, Universidade de Sao Paulo, Sao Paulo, SP, BR.
    Objectives:: Transcription Factor 21 represses steroidogenic factor 1, a nuclear receptor required for gonadal development, sex determination and the regulation of adrenogonadal steroidogenesis. The aim of this study was to investigate whether silencing or overexpression of the gene Transcription Factor 21 could modulate the gene and protein expression of steroidogenic factor 1 in adrenocortical tumors.

    Methods:: We analyzed the gene expression of steroidogenic factor 1 using qPCR after silencing endogenous Transcription Factor 21 in pediatric adrenal adenoma-T7 cells through small interfering RNA. Read More

    CONN SYNDROME PRESENTING AS CENTRAL RETINAL VEIN OCCLUSION.
    Retin Cases Brief Rep 2017 Jun 20. Epub 2017 Jun 20.
    *Department of Ophthalmology, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia; †Department of Endocrinology, The Canberra Hospital, Canberra, Australia; and ‡Department of Ophthalmology, The Canberra Hospital, Canberra, Australia.
    Purpose: To describe a case of central retinal vein occlusion in a young patient presenting with symptomatic malignant hypertension because of Conn syndrome.

    Methods: Single interventional case report.

    Results: A 44-year-old man presented with a 1-day history of headache and vision loss in his right eye on a background of malignant hypertension. Read More

    Suppression of Forkhead Box Protein O1 (FOXO1) Transcription Factor May Promote Adrenocortical Tumorigenesis.
    Horm Metab Res 2017 Aug 22;49(8):631-637. Epub 2017 Jun 22.
    Yale Endocrine Neoplasia Laboratory, Yale School of Medicine, New Haven, Connecticut, USA.
    Despite recent comprehensive genetic analyses, molecular evidence for a pathophysiological continuum linking benign adrenocortical adenoma (ACA) and highly aggressive adrenocortical carcinoma (ACC) is still elusive. Using human tumor samples and the established ACC cell line SW-13, this study investigated potential regulatory roles for FOXO transcription factors, in modulating adrenocortical tumorigenesis. Adrenocortical tumor specimens (20 ACAs, 10 ACCs, and 9 normal adrenal tissue samples) obtained from 30 patients were analyzed for ubiquitously expressed FOXO transcription factors, FOXO1 and FOXO3 using qRT-PCR and immunohistochemistry. Read More

    Cushing Syndrome: Diagnostic Workup and Imaging Features, With Clinical and Pathologic Correlation.
    AJR Am J Roentgenol 2017 Jul;209(1):19-32
    1 Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030.
    Objective: Cushing syndrome (CS) is a constellation of clinical signs and symptoms resulting from chronic exposure to excess cortisol, either exogenous or endogenous. Exogenous CS is most commonly caused by administration of glucocorticoids. Endogenous CS is subdivided into two types: adrenocorticotropic hormone (ACTH) dependent and ACTH independent. Read More

    Adrenal tumour bigger than 5 cm - what could it be? An analysis of 139 cases.
    Endokrynol Pol 2017 12;68(4):411-415. 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;127(7):2739-2750. 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 Sep 8;177(3):251-256. Epub 2017 Jun 8.
    Department of EndocrinologyInstitute of Medicine, Sahlgrenska Academy, University of Gothenburg and Sahlgrenska University Hospital, Göteborg, 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).

    Methods: Patients with NFPA were followed between years 1997 and 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 6;68(4):416-421. Epub 2017 Jun 6.
    Department of Endocrinology and Diabetology, Nicolaus Copernicus University in Toruń, Collegium Medicum in Bydgoszcz, Poland.
    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.
    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 her gonadotropin level did not elevate in a 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 Aug 18;177(2):K7-K12. Epub 2017 May 18.
    Clinical Division of Endocrinology and MetabolismDepartment of Medicine III, 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 Jul 17;49(7):520-526. 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 Jun 12;69(2):127-133. 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 21;87(5):295-300. Epub 2017 Apr 21.
    Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.
    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 Jun 12;69(2):235-239. 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

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