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    Disseminated Pancreatic Neuroendocrine Neoplasm (NEN) with an Uncommon Localisation in the Central Nervous System. A Case Report.
    Pol J Radiol 2017 1;82:120-125. Epub 2017 Mar 1.
    Faculty of Medical Sciences, University of Warmia and Mazury in Olsztyn, Olsztyn, Poland; Department of Radiology, Clinical University Hospital in Olsztyn, Olsztyn, Poland.
    Background: Neuroendocrine neoplasms (NEN) are rare neoplasms that originate from neuroendocrine cells and are characterized by the potential of hormonal activity. Approximately 70% of these tumours are located in the gastrointestinal system (GI), followed by the bronchi, endocrine glands-like C cells of the thyroid (medullary carcinoma), the parasympathetic and sympathetic system (paragangliomas, pheochromocytoma) and other very rare locations. The prevalence of cerebral metastases in neuroendocrine tumours is estimated by various authors to be approximately 1. Read More

    Risk factors of post-operative severe hyperlactatemia and lactic acidosis following laparoscopic resection for pheochromocytoma.
    Sci Rep 2017 Mar 24;7(1):403. Epub 2017 Mar 24.
    Department of Anesthesiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, P.R. China.
    Severe hyperlactatemia (SH)/lactic acidosis (LA) after laparoscopic resection of pheochromocytoma is an infrequently reported complication. The study aims to investigate the incidence of this complication and to determine the clinical risk factors. Patients who underwent laparoscopic resection for pheochromocytoma between 2011 and 2014 at Peking Union Medical College Hospital were enrolled. Read More

    Hypothalamo‑hypophysial system in rats with autotransplantation of the adrenal cortex.
    Mol Med Rep 2017 Mar 24. Epub 2017 Mar 24.
    Department of Anatomy and Cell Science, Kansai Medical University, Hirakata, Osaka 573‑1010, Japan.
    Patients with bilateral pheochromocytoma often require an adrenalectomy. Autotransplantation of the adrenal cortex is an alternative therapy that could potentially be performed instead of receiving glucocorticoid replacement following adrenalectomy. Adrenal cortex autotransplantation aims to avoid the side effects of long‑term steroid treatment and adrenal insufficiency. Read More

    Pheochromocytoma with Negative Metanephrines: A Rarity and the Significance of Dopamine Secreting Tumors.
    Urol Case Rep 2017 May 21;12:51-53. Epub 2017 Mar 21.
    Department of Urology, Ballarat Health Services, Victoria, Australia.
    We describe a case of a 25-year-old female with a dopamine secreting PPGL diagnosed retrospectively with biochemical analysis. This finding resulted in change in approach to investigation and management, given their important clinical implications. There are important differences in management of dopamine secreting PPGL compared to classical noradrenaline and adrenaline-secreting PPGL. Read More

    Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes.
    J Nucl Med 2017 Mar 23. Epub 2017 Mar 23.
    National Institute of Health, United States.
    Rationale: Pheochromocytoma/paraganglioma (PPGL) syndromes associated with polycythemia have previously been described in association with mutations in the von Hippel-Lindau (VHL) gene. Recently, mutations in the prolyl hydroxylase gene (PHD) 1 and 2 and in the hypoxia-inducible factor 2 alpha (HIF2A) were also found to be associated with multiple and recurrent PPGL. Such patients also presented with PPGL and polycythemia, and later on, some presented with duodenal somatostatinoma. Read More

    PRECISION MEDICINE: AN UPDATE ON GENOTYPE-BIOCHEMICAL PHENOTYPE RELATIONSHIPS IN PHEOCHROMOCYTOMA/PARAGANGLIOMA PATIENTS.
    Endocr Pract 2017 Mar 23. Epub 2017 Mar 23.
    From: Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA.
    Pheochromocytomas and paragangliomas are rare neuroendocrine tumors known to produce and secrete high levels of circulating catecholamines and their metabolites. The biochemical characteristics of these tumors can be used to divide them into three major phenotypes. The adrenergic phenotype comprises of tumors with predominant elevations in epinephrine and metanephrine levels whereas the noradrenergic phenotype is composed of tumors with predominant elevations in norepinephrine and normetanephrine levels. Read More

    An unusual diagnosis for persistent diarrhoea and vomiting.
    Gastroenterol Hepatol Bed Bench 2017 ;10(1):73-74
    Department of Gastroenterology, Milton Keynes University Hospital, Milton Keynes, UK. 2016.
    Identifying the etiology of chronic diarrhoea might be challenging in some patients, and before a diagnosis is made these patients may spend a substantial length of time with unresolved symptoms leading to uncertainty and anxiety that is severely impairing their life quality. A 45-year-old female was referred by her general practitioner with a 5-year history of increasingly frequent episodes of cyclical diarrhoea, vomiting, abdominal pain and intermittent palpitations. Contrast CT Abdomen/Pelvis revealed a 36x33x46 mm mass in the aorto caval region of her retro-peritoneum, just above the bifurcation. 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

    Global DNA Methylation Analysis Identifies Two Discrete clusters of Pheochromocytoma with Distinct Genomic and Genetic Alterations.
    Sci Rep 2017 Mar 22;7:44943. Epub 2017 Mar 22.
    Department of Surgical Sciences, Uppsala University, Uppsala, Sweden.
    Pheochromocytomas and paragangliomas (PPGLs) are rare and frequently heritable neural-crest derived tumours arising from the adrenal medulla or extra-adrenal chromaffin cells respectively. The majority of PPGL tumours are benign and do not recur with distant metastases. However, a sizeable fraction of these tumours secrete vasoactive catecholamines into the circulation causing a variety of symptoms including hypertension, palpitations and diaphoresis. Read More

    Assessment of clinical and radiologic differences between small and large adrenal pheochromocytomas.
    Clin Imaging 2017 Mar 10;43:153-157. Epub 2017 Mar 10.
    Department of Radiology, Dong-A University College of Medicine, Daesingongwon-ro 26, Seo-gu, Busan 602-715, South Korea.
    Objective: To evaluate differences in clinical and radiologic features of small and large pheochromocytomas.

    Materials And Methods: This study included 39 patients with adrenal pheochromocytomas. Several clinical and radiologic features were statistically analyzed and compared between small and large pheochromocytomas. Read More

    Risk profile of the RET A883F germline mutation: an international collaborative study.
    J Clin Endocrinol Metab 2017 Mar 17. Epub 2017 Mar 17.
    Endocrine Practice, Moleculargenetic Laboratory, Brückenstr 21, 69120 Heidelberg, Germany.
    Context: The A883F germline mutation of the REarranged during Transfection proto-oncogene causes multiple endocrine neoplasia 2B. In the revised American Thyroid Association (ATA) guidelines for the management of medullary thyroid carcinoma (MTC) the A883F mutation has been reclassified from the highest to high risk level, although no well-defined risk profile for this mutation exists.

    Objective: To create a risk profile for the A883F mutation for appropriate classification in the ATA risk levels. Read More

    The Histological Features of a Myocardial Biopsy Specimen in a Patient in the Acute Phase of Reversible Catecholamine-induced Cardiomyopathy due to Pheochromocytoma.
    Intern Med 2017 17;56(6):665-671. Epub 2017 Mar 17.
    Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan.
    A 63-year-old Japanese woman with an adrenal tumor was transferred to our hospital due to cardiogenic shock. Right and left ventriculography showed severe hypokinesis of the middle segment and the apex in both ventricles, and an endomyocardial biopsy demonstrated a small number of necrotic myocytes and cellular infiltration. She was diagnosed with pheochromocytoma and quickly recovered after treatment with an α-blocker. Read More

    Reassessing the clinical spectrum associated with Hereditary Leiomyomatosis and Renal Cell Carcinoma syndrome in French FH mutation carriers.
    Clin Genet 2017 Mar 16. Epub 2017 Mar 16.
    Département de Médecine Oncologique, Gustave Roussy, Université Paris-Saclay, Villejuif, F-94805, France.
    We addressed uncertainties regarding Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) by exploring all French cases, representing the largest series to date. Fumarate Hydratase (FH) germline testing was performed with Sanger sequencing and qPCR/MLPA. Enzyme activity was measured when necessary. Read More

    A Family with Von Hippel-Lindau Syndrome: The Findings of Indium-111 Somatostatin Receptor Scintigraphy, Iodine-123 Metaiodobenzylguanidine Scintigraphy and Single Photon Emission Computerized Tomography.
    Mol Imaging Radionucl Ther 2017 Feb;26(1):38-42
    Ankara Numune Training and Research Hospital, Clinic of Nuclear Medicine, Ankara, Turkey Phone: +90 542 286 75 75 E-mail:
    Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant hereditary familial disorder characterized by development of malignant and benign neoplasms. Differential diagnosis of the adrenal and pancreatic masses are difficult in patients with VHLS. Iodine-123 metaiodobenzylguanidine (I-123 MIBG) and indium-111 somatostatin receptor scintigraphies (In-111 SRS) have important roles in the differential diagnosis of adrenal and pancreatic masses in those patients. Read More

    Catecholamine-Induced Chest Pain Mimicking Infarction Due to an MIBG-Negative and DOPA-Positive Succinate Dehydrogenase Syndrome Subunit B-Related Pheochromocytoma.
    Clin Nucl Med 2017 Mar 10. Epub 2017 Mar 10.
    From the Departments of *Internal Medicine, and †Nuclear Medicine, Radiology, Neuroradiology, Medical Physics, Clinical Laboratory, Microbiology, Pathology, Molecular Biology, "Santa Maria della Misericordia" Hospital, Rovigo, Italy; and ‡Department of Radiology, University of Southern California, Los Angeles, CA.
    This 16-year-old boy presented with acute retrosternal pain possibly representing acute myocardial infarction. Cardiac enzymes were within reference ranges. There were marked increases in metanephrine to 3299 μg/24 h (reference, <400 μg/24 h), normetanephrine to 1309 μg/24 h (reference, 0-390 μg/24 h), and chromogranin A to 1605 ng/mL (reference, 0-150 μg/24 h). Read More

    [Composite pheochromocytoma: A rare adrenal tumor].
    Ann Pathol 2017 Mar 9. Epub 2017 Mar 9.
    Service d'anatomie et cytologie pathologiques, CHU Pontchaillou, 2, rue Henri-le-Guilloux, 35033 Rennes cedex 9, France.
    Introduction: Composite pheochromocytoma is a rare tumor of the adrenal medulla composed of pheochromocytoma and neuroblastic tumor. We report the case of a composite pheochromocytoma detected in a patient with neurofibromatosis type 1.

    Case Report: A 61-year-old male patient presented occasional sweats with palpitation and moderate high blood pressure. Read More

    Hypoxia, pseudohypoxia and cellular differentiation.
    Exp Cell Res 2017 Mar 8. Epub 2017 Mar 8.
    Translational Cancer Research, Lund University Cancer Center at Medicon Village, Lund University, Lund, Sweden. Electronic address:
    Tumor hypoxia correlates to aggressive disease, and while this is explained by a variety of factors, one clue to understand this phenomena was the finding that hypoxia induces a de-differentiated, stem cell-like phenotype in neuroblastoma and breast tumor cells. The hypoxia inducible transcription factors (HIFs) are regulated at the translational level by fluctuating oxygen concentrations, but emerging data reveal that both HIF-1α and HIF-2α expression can be induced by aberrantly activated growth factor signaling independently of oxygen levels. Furthermore, HIF-2α is regulated by hypoxia also at the transcriptional level in neuroblastoma and glioma cells. Read More

    Protective effect of medicinal fungus Xylaria nigripes mycelia extracts against hydrogen peroxide-induced apoptosis in PC12 cells.
    Int J Immunopathol Pharmacol 2017 Mar 1;30(1):105-112. Epub 2017 Feb 1.
    Department of Food and Nutrition, Providence University, Taichung, Republic of China (Taiwan).
    Xylaria nigripes ( XN) is a medicinal fungus that was used traditionally as a diuretic, nerve tonic, and for treating insomnia and trauma. In this study, we elucidated possible mechanisms of neuroprotective effects of XN mycelia extracts. XN mycelia were produced by fermentation. Read More

    Assessment of depression, anxiety, quality of life and coping in long-standing multiple endocrine neoplasia type 2 patients.
    Thyroid 2017 Mar 9. Epub 2017 Mar 9.
    Universidade de Sao Paulo Faculdade de Medicina, 37884, Endocrine Genetics Unit, Endocrinology Division , Av. Dr. Arnaldo, 455, 5º floor , Sao Paulo, Sao Paulo, Brazil , 01246-903.
    Background: Scarce data on psychological harm in multiple endocrine neoplasia type 2 (MEN2) are available.

    Objectives: To assess anxiety, depression, quality of life and coping in long-standing MEN2 patients.

    Patients And Methods: Forty-three adults (age ≥ 18 y) with clinical and genetic diagnosis of MEN2 and long-term follow-up (10. Read More

    Neurotoxicity induced by methamphetamine-heroin combination in PC12 cells.
    Neurosci Lett 2017 Mar 6;647:1-7. Epub 2017 Mar 6.
    Wuhan Institutes of Biomedical Sciences, Jianghan University, Wuhan 430056, China. Electronic address:
    Simultaneous administration of psychostimulants and opioids is a major drug abuse problem worldwide. The combination of psychostimulants and opioids produces more serious effects than either drug alone and is responsible for numerous deaths. In recent years, owing to its increased use, methamphetamine (METH), a psychostimulant, has become a popular choice for use in combination with opioids, especially heroin. Read More

    Identification of dibenzyl imidazolidine and triazole acetamide derivatives through virtual screening targeting amyloid beta aggregation and neurotoxicity in PC12 cells.
    Eur J Med Chem 2017 Apr 28;130:354-364. Epub 2017 Feb 28.
    Discipline of Pharmacology, Adelaide Medical School, Faculty of Health Sciences, The University of Adelaide, South Australia, Australia. Electronic address:
    Aggregation and neurotoxicity of amyloid β (Aβ) protein is a hallmark characteristic of Alzheimer's disease (AD). In this study we compared the anti-aggregatory and neuroprotective effects of five synthetic compounds against Aβ protein; four of which possessed a five membered heterocycle ring scaffold (two dibenzyl phenyl imidazolidines and two triazole sulfanyl acetamides) and one with a fused five membered heterocycle (benzoxazole) ring, selected thorough virtual screening from ZINC database. Molecular docking of their optimized structures was used to study Aβ binding characteristics. Read More

    Emerging role of dopamine in neovascularization of pheochromocytoma and paraganglioma.
    FASEB J 2017 Mar 6. Epub 2017 Mar 6.
    Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
    Dopamine is a catecholamine that acts both as a neurotransmitter and as a hormone, exerting its functions via dopamine (DA) receptors that are present in a broad variety of organs and cells throughout the body. In the circulation, DA is primarily stored in and transported by blood platelets. Recently, the important contribution of DA in the regulation of angiogenesis has been recognized. Read More

    Primary Clear Cell Microcystic Adenoma of the Sinonasal Cavity: Pathological or Fortuitous Association?
    Case Rep Pathol 2017 5;2017:9236780. Epub 2017 Feb 5.
    University of Southampton, Southampton, UK; Wessex Clinical Genetics Service, University Hospitals Southampton NHS Foundation Trust, Southampton, UK.
    Primary clear cell microcystic adenoma of the sinonasal cavity is rare. It has previously been described only as a VHL-associated tumour. Von Hippel-Lindau (VHL) syndrome is an inherited cancer syndrome characterised by an elevated risk of neoplasia including clear cell renal cell carcinoma (ccRCC), haemangioblastoma, and phaeochromocytoma. Read More

    Protective effect of arctigenin on ethanol-induced neurotoxicity in PC12 cells.
    Mol Med Rep 2017 Feb 21. Epub 2017 Feb 21.
    College of Pharmacy and Bioengineering, Chongqing University of Technology, Chongqing 400054, P.R. China.
    As a neurotropic substance, ethanol can damage nerve cells through an increase in the production of free radicals, interference of neurotrophic factor signaling pathways, activation of endogenous apoptotic signals and other molecular mechanisms. Previous studies have revealed that a number of natural drugs extracted from plants offer protection of nerve cells from damage. Among these, arctigenin (ATG) is a lignine extracted from Arctium lappa (L. Read More

    Impact of Surgical Resection of the Primary Tumor on Overall Survival in Patients With Metastatic Pheochromocytoma or Sympathetic Paraganglioma.
    Ann Surg 2017 Mar 2. Epub 2017 Mar 2.
    *Department of Endocrinology, Hospital Universitario San Vicente Fundación-Universidad de Antioquia, Medellín, Colombia †Department of Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, TX ‡Department of Biostatistics, University of Texas, MD Anderson Cancer Center, Houston, TX §Department of Health Services Research, University of Texas, MD Anderson Cancer Center, Houston, TX ¶Department of Urology, University of Texas MD Anderson Cancer Center, Houston, TX ||Department of Surgical Oncology, University of Texas, MD Anderson Cancer Center, Houston, TX.
    Objective: To determine whether primary tumor resection in patients with metastatic pheochromocytoma or paraganglioma (PPG) is associated with longer overall survival (OS).

    Background: Patients with metastatic PPG have poor survival outcomes. The impact of surgical resection of the primary tumor on OS is not known. Read More

    Structured assessment and followup for patients with hereditary kidney tumour syndromes.
    Can Urol Assoc J 2016 Jul-Aug;10(7-8):E214-E222. Epub 2016 Jul 12.
    Section of Urology, Department of Surgery, University of Manitoba, Winnipeg, MB, Canada.
    Introduction: Optimal clinical assessment and subsequent followup of patients with or suspected of having a hereditary renal cell carcinoma syndrome (hRCC) is not standardized and practice varies widely. We propose protocols to optimize these processes in patients with hRCC to encourage a more uniform approach to management that can then be evaluated.

    Methods: A review of the literature, including existing guidelines, was carried out for the years 1985-2015. Read More

    Large adrenal leiomyoma presented as adrenal incidentaloma in an AIDS patient: A rare entity.
    Med J Malaysia 2017 Feb;72(1):65-67
    Hospital Sultan Ismail, Department of Surgery, Johor Bahru, Malaysia.
    The literature on adrenal gland tumour in HIV-infected patients is scarce. We report a 46-year-old Malay man with HIV and Hepatitis C infection presenting with a large nonfunctioning adrenal tumour. Computed tomography showed a large right adrenal tumour with heterogeneous enhancement and central necrosis. Read More

    The acute exposure of tetrachloro-p-benzoquinone (a.k.a. chloranil) triggers inflammation and neurological dysfunction via Toll-like receptor 4 signaling: The protective role of melatonin preconditioning.
    Toxicology 2017 Apr 24;381:39-50. Epub 2017 Feb 24.
    Key Laboratory of Luminescence and Real-Time Analytical Chemistry (Southwest University), Ministry of Education, College of Pharmaceutical Sciences, Southwest University, Chongqing, 400715, People's Republic of China. Electronic address:
    This study is aimed to investigate the inflammation and neurological dysfunction induced by tetrachloro-p-benzoquinone (TCBQ) through Toll-like receptor 4 (TLR4) signaling. We also investigated the protective role of melatonin as an antioxidant and anti-inflammatory agent. In vitro model was established by rat pheochromocytoma PC12 cells, meanwhile, TLR4 wild-type (C57BL/6) and knockout mice (C57BL/10ScNJ TLR4(-/-)) were used as in vivo model. Read More

    Malignant pheochromocytoma-paraganglioma: pathogenesis, TNM staging, and current clinical trials.
    Curr Opin Endocrinol Diabetes Obes 2017 Feb 23. Epub 2017 Feb 23.
    aDepartment of Endocrinology, Hospital Universitario San Vicente Fundacion-Universidad de Antioquia, Medellín, Colombia bDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
    Purpose Of Review: Pheochromocytomas and paragangliomas (PPGs) are rare neuroendocrine tumors. Over the last 15 years, substantial progress has been made toward understanding the clinical aspects and molecular origins of this disease. Nevertheless, predicting and managing malignancy remains the biggest challenge in clinical practice. Read More

    Implications of SDHB genetic testing in patients with sporadic pheochromocytoma.
    Langenbecks Arch Surg 2017 Feb 22. Epub 2017 Feb 22.
    Department of Nuclear Medicine, La Timone University Hospital, APHM, CERIMED, Aix Marseille Univ, Marseille, France.
    Purpose: Succinate dehydrogenase B (SDHB) associated pheochromocytomas (PHEOs) are associated with a higher risk of tumor aggressiveness and malignancy. The aim of the present study was to evaluate (1) the frequency of germline SDHB mutations in apparently sporadic patients with PHEO who undergo preoperative genetic testing and (2) the ability to predict pathogenic mutations.

    Methods: From 2012 to 2016, 82 patients underwent a PHEO surgical resection. Read More

    Surgical treatment of left ventricular wall rupture, regarded as a consequence of Takotsubo cardiomyopathy.
    SAGE Open Med Case Rep 2017 18;5:2050313X16689210. Epub 2017 Jan 18.
    Scientific Research Institute of Heart Surgery and Organ Transplantation, Bishkek, Kyrgyzstan.
    Objective: We present the case of possible reverse type of TCM in a female patient presented with progressive left ventricular dysfunction and its rupture in pericardium.

    Methods: The detailed history, physical examination, laboratory tests, electrocardiography, serial echocardiography, coronary angiography with left ventriculography were performed to diagnose possible Takotsubo cardiomyopathy in 63-year old woman admitted to our center with complaints of dyspnea, lightheadedness, weakness and signs of hypotension and history of inferior myocardial infarction, acute left ventricular aneurysm, and effusive pericarditis and pleuritis, developed after emotional stress 5 months ago.

    Results: Clinical evaluation revealed unremarkable laboratory tests, normal troponin values, signs of old inferior myocardial infarction on electrocardiogram, and left ventricular (LV) dilatation and dysfunction, akinesia of LV infero-lateral wall with thinning and its rupture and blood shunting in pericardium. Read More

    Update on CT and MRI of Adrenal Nodules.
    AJR Am J Roentgenol 2017 Feb 22:1-12. Epub 2017 Feb 22.
    2 Department of Radiology, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, PA.
    Objective: The objective of this article is to review the current role of CT and MRI for the characterization of adrenal nodules.

    Conclusion: Unenhanced CT and chemical-shift MRI have high specificity for lipid-rich adenomas. Dual-energy CT provides comparable to slightly lower sensitivity for the diagnosis of lipid-rich adenomas but may improve characterization of lipid-poor adenomas. Read More

    Changes in Body Mass Index in Pheochromocytoma Patients Following Adrenalectomy.
    Horm Metab Res 2017 Feb 21. Epub 2017 Feb 21.
    Medizinische Klinik und Poliklinik IV, Endocrine Research Unit, Klinikum der Universität München, LMU, Ziemssenstr. 1, Munich, Germany.
    Catecholamine excess from pheochromocytoma results in cardiovascular symptoms such as arterial hypertension and tachycardia and induces metabolic alterations including glucose intolerance and increase in resting metabolic rate. The objective of our study was to investigate the effect of surgical cure of pheochromocytoma on body-mass-index and the correlation of body-mass-index changes to preoperative endocrine parameters. Pheochromocytoma patients from the Munich ENSAT Registry were matched (1:2) for age and gender to patients from the German Conn's Registry, who had undergone surgery for aldosterone-producing-adenomas. Read More

    Study of awareness of adrenal disorders among interns and postgraduate students of Hamidia Hospital, Bhopal.
    Indian J Endocrinol Metab 2017 Jan-Feb;21(1):251-254
    Department of Medicine, Gandhi Medical College, Bhopal, Madhya Pradesh, India.
    Introduction: Adrenal disorders could be a life-threatening emergency, hence requires immediate therapeutic management. For this awareness regarding its diagnosis, management, and treatment is prime important.

    Aims And Objective: To study the awareness of adrenal disorders among interns and postgraduates students of Hamidia Hospital, Bhopal. Read More

    Bowel perforation complicating an ACTH-secreting phaeochromocytoma.
    Endocrinol Diabetes Metab Case Rep 2016 24;2016. Epub 2016 Aug 24.
    Austin Health, Heidelberg, Victoria, Australia; University of Melbourne, Parkville, Victoria, Australia.
    ACTH-secreting phaeochromocytoma (ASP) is a rare cause of ACTH-dependent Cushing's syndrome (CS). We report the case of a 63-year-old female presenting with CS secondary to an ASP complicated by bowel perforation. This case report highlights ASP as an uncommon but important cause of ectopic ACTH secretion (EAS). Read More

    Mixed corticomedullary adrenal carcinoma - case report: Comparison in features, treatment and prognosis with the other two reported cases.
    Int J Surg Case Rep 2017 31;31:254-261. Epub 2017 Jan 31.
    Alberoni Hospital, Damascus University Faculty of Medicine, P.O. Box: 60527, Damascus, Syria. Electronic address:
    Introduction: Adrenal corticomedullary adenoma was reviewed in many cases in PubMed Library, While the coincidence corticomedullary adrenal carcinoma in the same gland was just described in two cases in the medical literature. Our case is the third to be reported and was treated with surgery and adjuvant chemotherapy and followed for two years.

    Presentation Of Case: A 50-year-old man suffered from a mass effect in the left abdominal side. Read More

    Atypical presentation of pheochromocytoma: Central nervous system pseudovasculitis.
    Indian J Urol 2017 Jan-Mar;33(1):82-84
    Department of Urology and Kidney Transplant, Medanta - The Medicity, Gurgaon, Haryana, India.
    Pheochromocytoma has atypical presentation in 9%-10% of patients. Atypical presentations include myocardial infarction, renal failure, and rarely cerebrovascular events. Various etiologies for central nervous system (CNS) involvement in pheochromocytoma have been described in the literature. Read More

    Current perioperative management of pheochromocytomas.
    Indian J Urol 2017 Jan-Mar;33(1):19-25
    Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, New Delhi, India.
    Neuroendocrine tumors which have the potential to secrete catecholamines are either associated with sympathetic adrenal (pheochromocytoma) or nonadrenal (paraganglioma) tissue. Surgical removal of these tumors is always indicated to cure and prevent cardiovascular and other organ system complications associated with catecholamine excess. Some of these tumors have malignant potential as well. Read More

    [Clinical features and mutations of RET proto-oncogene in a pedigree affected with type 2A multiple endocrine neoplasia].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2017 Feb;34(1):106-109
    Department of Endocrinology and Metabolism, Huai'an Second People's Hospital, Huai'an, Jiangsu 223002, China.
    Objective: To investigate the clinical features and mutations of RET proto-oncogene in a pedigree affected with multiple endocrine neoplasia type 2A (MEN2A).

    Methods: Clinical data of the family members was collected. Genomic DNA from peripheral blood leukocytes were extracted and subjected to PCR amplification. Read More

    Recommendations for the management of adrenal incidentalomas: what is pertinent for radiologists?
    Br J Radiol 2017 Apr 9;90(1072):20160627. Epub 2017 Feb 9.
    Department of Imaging, St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.
    Adrenal incidentalomas are unsuspected, asymptomatic adrenal masses detected on imaging. Most are non-functioning benign adrenocortical adenomas but can represent other benign lesions or lesions requiring therapeutic intervention including adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis. This review summarizes and highlights radiological recommendations within the recently issued guidelines for the management of adrenal incidentalomas from the European Society of Endocrinology Clinical Practice in collaboration with the European Network for Study of Adrenal Tumours. Read More

    Potential Pitfalls of SDH Immunohistochemical Detection in Paragangliomas and Phaeochromocytomas Harbouring Germline SDHx Gene Mutation.
    Anticancer Res 2017 02;37(2):805-812
    Division of Pathological Anatomy, University of Florence, Florence, Italy
    Background/aim: Germline mutations in any of the succinate dehydrogenase (SDH) genes result in destabilization of the SDH protein complex and loss of SDHB expression at immunohistochemistry. SDHA is lost together with SDHB in SDHA-mutated tumours, but its expression is retained in tumours with other SDH mutations. We investigated whether SDHA/SDHB immunohistochemistry is able to identify SDH-related tumours in a retrospective case series of phaeochromocytomas (PCCs) and paragangliomas (PGLs). Read More

    Peptide Receptor Radionuclide Treatment and (131)I-MIBG in the management of patients with metastatic/progressive phaeochromocytomas and paragangliomas.
    J Surg Oncol 2017 Feb 6. Epub 2017 Feb 6.
    ENETS Centre of Excellence Neuroendocrine Tumour Unit, Royal Free London NHS Foundation Trust, London, UK.
    Background And Objectives: Radionuclide therapy has been used to treat patients with progressive/metastatic paragangliomas (PGLs) and phaeochromocytomas (PCCs). The aim of the present study is to retrospectively compare the therapeutic outcomes of these modalities in patients with progressive/metastatic PCCs and PGLs.

    Methods: Patients with progressive/metastatic PGLs and PCCs that were subjected to radionuclide treatment in our department were retrieved from our department's database for the period 1998-2013. Read More

    Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma.
    Cancer Cell 2017 Feb 2;31(2):181-193. Epub 2017 Feb 2.
    Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA; Department of Genetics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. Electronic address:
    We report a comprehensive molecular characterization of pheochromocytomas and paragangliomas (PCCs/PGLs), a rare tumor type. Multi-platform integration revealed that PCCs/PGLs are driven by diverse alterations affecting multiple genes and pathways. Pathogenic germline mutations occurred in eight PCC/PGL susceptibility genes. Read More

    Biomarker, Molecular, and Technologic Advances in Urologic Pathology, Oncology, and Imaging.
    Arch Pathol Lab Med 2017 Feb 3. Epub 2017 Feb 3.
    From the Departments of Pathology (Drs Ellis, Harik, Cohen, and Osunkoya), Urology (Dr Osunkoya), and the Winship Cancer Institute (Dr Osunkoya), Emory University School of Medicine, Atlanta, Georgia; and the Department of Pathology, Veterans Affairs Medical Center, Atlanta, Georgia (Dr Osunkoya).
    Urologic pathology is evolving rapidly. Emerging trends include the expanded diagnostic utility of biomarkers and molecular testing, as well as adapting to the plethora of technical advances occurring in genitourinary oncology, surgical practice, and imaging. We illustrate those trends by highlighting our approach to the diagnostic workup of a few selected disease entities that pathologists may encounter, including newly recognized subtypes of renal cell carcinoma, pheochromocytoma, and prostate cancer, some of which harbor a distinctive chromosomal translocation, gene loss, or mutation. Read More

    Adrenomedullin - new perspectives of a potent peptide hormone.
    J Pept Sci 2017 Feb 2. Epub 2017 Feb 2.
    Faculty of Biosciences, Pharmacy and Psychology, Institute of Biochemistry, Leipzig University, Brüderstraße 34, 04103, Leipzig, Germany.
    Adrenomedullin (ADM) is a 52-amino acid multifunctional peptide, which belongs to the calcitonin gene-related peptide (CGRP) superfamily of vasoactive peptide hormones. ADM exhibits a significant vasodilatory potential and plays a key role in various regulatory mechanisms, predominantly in the cardiovascular and lymphatic system. It exerts its effects by activation of the calcitonin receptor-like receptor associated with one of the receptor activity-modifying proteins 2 or 3. Read More

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