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    Single-stage laparoscopic adrenalectomy for pheochromocytoma and enucleation of a pancreatic neuroendocrine tumor in Von Hippel-Lindau disease: A case report.
    Mol Clin Oncol 2017 May 6;6(5):799-801. Epub 2017 Apr 6.
    Department of Surgery, IRCCS - Azienda Ospedaliera Universitaria San Martino-IST, University of Genoa, Ι-16132 Genoa, Italy.
    Von Hippel-Lindau (VHL) disease is an inherited syndrome with autosomal-dominant transmission, characterized by central nervous system and retinal hemangioblastomas, visceral cysts and tumors. Optimal surgical treatment, including its timing, remains a controversial topic. The present study reports the case of a 67-year-old female patient with adrenal and pancreatic manifestations of VHL. Read More

    Diagnostic Imaging of Pregnant Women - The Role of Magnetic Resonance Imaging.
    Pol J Radiol 2017 19;82:220-226. Epub 2017 Apr 19.
    Clinic of Obstetrics and Gynecology, Institute of Mother and Child, Warsaw, Poland.
    Background: Presentation of magnetic resonance imaging (MRI) findings in pregnant women in the Department of Diagnostic Imaging, Institute of Mother and Child, Warsaw, Poland.

    Material/methods: Forty-three symptomatic pregnant women underwent MRI between 9 and 33 weeks of gestation (mean of 23 weeks). Moreover, we included 2 pregnant women who underwent fetal MRI and had incidental abnormalities. Read More

    Application of Panel-Based Tests for Inherited Risk of Cancer.
    Annu Rev Genomics Hum Genet 2017 May 15. Epub 2017 May 15.
    Division of Translational Medicine and Human Genetics, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104; email:
    Next-generation or massively parallel sequencing has transformed the landscape of genetic testing for cancer susceptibility. Panel-based genetic tests evaluate multiple genes simultaneously and rapidly. Because these tests are frequently offered in clinical settings, understanding their clinical validity and utility is critical. Read More

    The Penetrance of Paraganglioma and Pheochromocytoma in SDHB germline mutation carriers.
    Clin Genet 2017 May 14. Epub 2017 May 14.
    Department of Otorhinolaryngology - Head Neck Surgery, VU University Medical Center, Amsterdam, the Netherlands.
    Germline mutations in SDHB predispose to hereditary paraganglioma syndrome type 4. The risk of developing paraganglioma (PGL) or pheochromocytoma (PHEO) in SDHB mutation carriers is subject of recent debate. In the present nationwide cohort study of SDHB mutation carriers identified by the clinical genetics centers of the Netherlands, we have calculated the penetrance of SDHB associated tumors using a novel maximum likelihood estimator. Read More

    The dynamics of early-state transcriptional changes and aggregate formation in a Huntington's disease cell model.
    BMC Genomics 2017 May 12;18(1):373. Epub 2017 May 12.
    Synthetic, Systems Biology and Nuclear Organization, Swammerdam Institute for Life Sciences, University of Amsterdam, Amsterdam, The Netherlands.
    Background: Huntington's disease (HD) is a fatal neurodegenerative disorder caused by a CAG expansion in the Huntingtin (HTT) gene. Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. To understand the time-resolved relationship between aggregate formation and transcriptional changes at early disease stages, we performed temporal transcriptome profiling and quantification of aggregate formation in living cells in an inducible HD cell model. Read More

    Luteolin-induced apoptosis through activation of endoplasmic reticulum stress sensors in pheochromocytoma cells.
    Mol Med Rep 2017 May 12. Epub 2017 May 12.
    Department of Anatomy and Cell Biology, College of Medicine, Chungnam National University, Daejeon 35015, Republic of Korea.
    Luteolin [2-(3,4-dihydroxyphenyl)-5,7-dihydroxy-4-chromenone] is an active flavonoid compound from Lonicera japonica (Caprifoliaceae). Luteolin inhibits tumor cell proliferation, inflammatory and oxidative stress better, when compared with other flavonoids. In the present study, it was demonstrated that luteolin induces typical apoptosis in PC12 cells (derived from a pheochromocytoma of the rat adrenal medulla) accompanied by DNA fragmentation and formation of apoptotic bodies. 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

    Extra-adrenal pheochromocytoma at the organ of Zuckerkandl: a case report and literature review.
    Radiol Case Rep 2017 Jun 21;12(2):343-347. Epub 2017 Mar 21.
    Diagnostic Radiology, University of Missouri-Kansas City, Kansas City, MO, USA.
    Pheochromocytomas and paragangliomas are tumors that occur in characteristic locations and are commonly detected on imaging studies. A correct diagnosis is important because of differences in associated neoplasms, risk for malignancy, and need for genetic testing. In addition, associated complications, including death, can be avoided if appropriately recognized and treated. Read More

    The Phenotype of SDHB Germline Mutation Carriers; a Nationwide Study.
    Eur J Endocrinol 2017 May 10. Epub 2017 May 10.
    E Corssmit, Endocrinology, Leiden University Medical Center, Leiden, Netherlands.
    Objective Succinate dehydrogenase B subunit (SDHB) gene germline mutations predispose to pheochromocytomas, sympathetic paragangliomas, head and neck paragangliomas, and non-paraganglionic tumors (e.g. renal cell carcinoma, gastrointestinal stromal tumor and pituitary neoplasia). Read More

    Isoliquiritigenin Induces Cytotoxicity in PC-12 Cells In Vitro.
    Appl Biochem Biotechnol 2017 May 9. Epub 2017 May 9.
    School of Pharmacy, Guangdong Pharmaceutical University, Guangzhou, 510006, People's Republic of China.
    Isoliquiritigenin (ISL) has been reported to have a wide range of biological activities. This study evaluated the cytotoxic effect of ISL on norvegicus pheochromocytoma cell line (PC-12 cells) and its possible molecular mechanism. The cytotoxicity in vitro of ISL against PC-12 cells was investigated by MTT assay. Read More

    Comparison of the 68Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT in the Evaluation of Suspected Primary Pheochromocytomas and Paragangliomas.
    Clin Nucl Med 2017 May 6. Epub 2017 May 6.
    From the *Department of Nuclear Medicine, Peking Union Medical College (PUMC) Hospital, Chinese Academy of Medical Science & PUMC, Beijing; †Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing; and ‡Department of Endocrinology, PUMC Hospital, Chinese Academy of Medical Science & PUMC, Beijing, China.
    Anatomical imaging modalities including CT and MRI are the mainstay of evaluation of primary pheochromocytoma or paraganglioma. However, nuclear medicine imaging is frequently necessary to determine the nature of the lesions. The purpose of this investigation is to assess which commonly used nuclear medicine modality might have a better diagnostic value in this clinical setting. Read More

    Development and validation of a liquid chromatography tandem mass spectrometry method for the measurement of urinary catecholamines in diagnosis of pheochromocytoma.
    Biomed Chromatogr 2017 May 8. Epub 2017 May 8.
    Department of Laboratory Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, P.R. China.
    The measurement of catecholamines in human body fluids is requested frequently for the differential diagnosis and monitoring of pheochromocytoma. The methods in most clinical laboratories focus on high performance liquid chromatography coupled with electrochemical detection which suffers from high background noise, low sensitivity, and poor separation. We reported and developed a robust high-throughput liquid chromatography tandem mass spectrometry method in routine clinical laboratories for the measurement of urinary catecholamines for diagnosis of pheochromocytoma. 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

    Routine genetic screening with a multi-gene panel in patients with pheochromocytomas.
    Endocrine 2017 May 5. Epub 2017 May 5.
    Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK, OX3 7LE, UK.
    Purpose: Several new gene mutations have been reported in recent years to be associated with a risk of familial pheochromocytoma. However, it is unclear as to whether extensive genetic testing is required in all patients.

    Methods: The clinical data of consecutive patients operated for pheochromocytoma over a decade in a tertiary referral center were reviewed. Read More

    Plasma Methoxytyramine: Clinical Utility with Metanephrines for Diagnosis of Pheochromocytoma and Paraganglioma.
    Eur J Endocrinol 2017 May 5. Epub 2017 May 5.
    G Eisenhofer, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
    Context: Measurements of plasma methoxytyramine, the O-methylated dopamine metabolite, are useful for detecting rare dopamine-producing pheochromocytomas and paragangliomas (PPGLs) and head and neck paragangliomas (HNPGLs), but utility for screening beyond that achieved using standard measurements of normetanephrine and metanephrine is unclear.

    Objective: Evaluation of the additional utility of methoxytyramine compared to plasma normetanephrine and metanephrine for diagnosis of PPGLs and HNPGLs.

    Design: Comparative prospective study Methods: Comparison of mass spectrometric-based measurements of plasma methoxytyramine, normetanephrine and metanephrine in 1963 patients tested for PPGLs at six tertiary medical centers according to reference intervals verified in 423 normotensive and hypertensive volunteers. Read More

    Mitochondrial Deficiencies in the Predisposition to Paraganglioma.
    Metabolites 2017 May 4;7(2). Epub 2017 May 4.
    INSERM UMR970, Paris-Cardiovascular Research Center at HEGP, F-75015 Paris, France.
    Paragangliomas and pheochromocytomas are rare neuroendocrine tumours with a very strong genetic component. It is estimated that around 40% of all cases are caused by a germline mutation in one of the 13 predisposing genes identified so far. Half of these inherited cases are intriguingly caused by mutations in genes encoding tricarboxylic acid enzymes, namely SDHA, SDHB, SDHC, SDHD, and SDHAF2 genes, encoding succinate dehydrogenase and its assembly protein, FH encoding fumarate hydratase, and MDH2 encoding malate dehydrogenase. Read More

    Recurrence of non-cardiogenic pulmonary edema and sustained hypotension shock in cystic pheochromocytoma.
    J Zhejiang Univ Sci B 2017 May;18(5):449-452
    Department of Cardiology, Zhejiang Provincial Hospital of Traditional Chinese Medicine, Hangzhou 310006, China.
    Pheochromocytoma is a rare neuroendocrine tumor which derives from chromaffin cells of the adrenal gland or relevant to sympathetic nerves and ganglia. The clinical features of pheochromocytoma are various. Paroxysmal episodes of serious hypertension, headache, palpitation, and diaphoresis are the typical manifestations (Bravo, 2004). Read More

    β-N-Methylamino-L-Alanine Toxicity in PC12: Excitotoxicity vs. Misincorporation.
    Neurotox Res 2017 May 3. Epub 2017 May 3.
    Department of Biochemistry and Microbiology, Nelson Mandela Metropolitan University, P.O. Box 77000, Port Elizabeth, 6031, South Africa.
    The implication of β-N-methylamino-L-alanine (BMAA) in the development of neurodegenerative diseases worldwide has led to several investigations of the mechanism, or mechanisms, of toxicity of this cyanobacterially produced amino acid. The primary mechanism of toxicity that was identified is excitotoxicity, with a second possible mechanism, the misincorporation of BMAA into the primary protein structure and consequent cell damage, having been more recently reported. However, studies on excitotoxicity and misincorporation have been conducted independently and there are therefore no data available on the relative contribution of each of these mechanisms to the total toxicity of BMAA. Read More

    18-Fluoro-deoxyglucose uptake in inflammatory hepatic adenoma: A case report.
    World J Hepatol 2017 Apr;9(11):562-566
    Willy Liu, Olivier Detry, Department of Abdominal Surgery and Transplantation, CHU Liege (CHU-ULg), B4000 Liege, Belgium.
    Positron emission tomography computed tomography (PET-CT) using 18-Fluoro-deoxyglucose ((18)FDG) is an imaging modality that reflects cellular glucose metabolism. Most cancers show an uptake of (18)FDG and benign tumors do not usually behave in such a way. The authors report herein the case of a 38-year-old female patient with a past medical history of cervical intraepithelial neoplasia and pheochromocytoma, in whom a liver lesion had been detected with PET-CT. Read More

    Genotype-Phenotype Correlation in Patients With Germline Mutations of VHL, RET, SDHB, and SDHD Genes: Thai Experience.
    Clin Med Insights Endocrinol Diabetes 2017 20;10:1179551417705122. Epub 2017 Apr 20.
    Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
    Mutations in the VHL, RET, SDHB, and SDHD genes are responsible for von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia type 2 (MEN2), and familial paraganglioma, respectively. However, genotype-phenotype correlation data are lacking in Southeast Asia. A retrospective medical chart review was performed on patients referred to the genetics service. Read More

    Pheochromocytoma in a Child without Hypertension: A Contribution to the "Rule of 10s".
    Indian J Med Paediatr Oncol 2017 Jan-Mar;38(1):59-61
    Department of Pediatric Surgery, SCB Medical College, Cuttack, Odisha, India.
    Pheochromocytoma (PCC) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla. Its diagnosis and treatment are well defined in adults, but experience in children is limited. Children constitute only 10% of reported cases, the average age at presentation being 11 years. Read More

    Examination of PHOX2B in Adult Neuroendocrine Neoplasms Reveals Relatively Frequent Expression in Pheochromocytomas and Paragangliomas.
    Histopathology 2017 May 2. Epub 2017 May 2.
    Department of Pathology, University of Iowa Hospitals and Clinics and Carver College of Medicine, Iowa City, IA, USA.
    Aims: Paired-like homeobox 2b (PHOX2B) is a transcription factor with expression outside of the central nervous system restricted to neurons and chromaffin cells of the autonomic nervous system. Germline mutations cause congenital central hypoventilation syndrome and predispose to neuroblastoma and Hirschsprung disease. Among pediatric small round cell tumors, PHOX2B is neuroblastoma-specific. Read More

    Complete laparoscopic excision of a giant retroperitoneal paraganglioma.
    Ann R Coll Surg Engl 2017 May;99(5):e148-e150
    HPB Surgery and Dept of Cardiology and Endocrinology, Hammersmith Hospital, Imperial College Healthcare Trust, Du Cane Road , London , UK.
    Paragangliomas (or 'extra-adrenal phaeochromocytomas') are rare tumours arising from neural crest cells. They occur most commonly in the abdomen along the paraspinal sympathetic ganglion. The malignancy rate can be up to 35% and surgical resection is the recommended treatment. Read More

    Multiple Recurrent Paraganglioma in a Pediatric Patient with Germline SDH-B Mutation.
    Urol Case Rep 2017 Jul 27;13:107-109. Epub 2017 Apr 27.
    National Cancer Institute, National Institutes of Health, Urologic Oncology Branch, Bethesda, MD, USA.
    Magnetic Resonance Imaging (MRI) and fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) are recognized approaches for locating paragangliomas. Recently, gallium-68 DOTA-octreotate (DOTATATE) scans have shown promise detecting neuroendocrine tumors missed by FDG-PET and MRI. 13-year-old male with SDH-B mutation presented with symptoms of paraganglioma and elevated catecholamines. Read More

    Novel germline variant of TMEM127 gene in a patient with familial pheochromocytoma.
    Endocrinol Diabetes Metab Case Rep 2017 6;2017. Epub 2017 Apr 6.
    Departments of Diabetes and Endocrinology.
    Summary: Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumours with a heterogeneous genetic background. Up to 40% of apparently sporadic PCC/PGL cases carry 1 of the 12 gene germline mutations conferring genetic susceptibility to PCC/PGL. Although the precise mechanisms are unclear, TMEM127 is one of the rare responsible genes for PCC/PGL. Read More

    An unusual cause of back pain: a case of large nonfunctioning retroperitoneal paraganglioma presented as a large cystic lesion. A case report and review of literature.
    J Surg Case Rep 2017 Apr 21;2017(4):rjx059. Epub 2017 Apr 21.
    Department of Radiology, United Christian Hospital, Hong Kong.
    Pheochromocytoma arising from outside the adrenal glands is also called paraganglioma. When it occurs below the diaphragm, in the organ of Zuckerkandl or retroperitoneum, it is also called extra-adrenal pheochromocytoma. Paragangliomas are rare tumors which arise from neuroendocrine cells and extra-adrenal paragangliomas (EAPs) account for only 10-15% if all paragangliomas and may present incidentally as a symptomless mass. Read More

    NVP-AUY922, a novel HSP90 inhibitor, inhibits the progression of malignant pheochromocytoma in vitro and in vivo.
    Onco Targets Ther 2017 19;10:2219-2226. Epub 2017 Apr 19.
    Department of Urology, Ruijin Hospital.
    Purpose: Malignant pheochromocytoma (PCC) is a rare tumor with a very poor prognosis and no effective treatments. The aim of this study was to assess the efficacy of a novel second-generation synthetic heat-shock protein 90 (HSP90) inhibitor, NVP-AUY922, to treat malignant PCC in vitro and in vivo.

    Materials And Methods: Cell Counting Kit-8 (CCK-8) and Transwell assays were used to assess the effects of NVP-AUY922 on the proliferation and migration of the PCC cell line PC12. Read More

    Surgical approaches in hereditary endocrine tumors.
    Updates Surg 2017 Apr 28. Epub 2017 Apr 28.
    Minimally Invasive Endocrine Surgery Unit, Department of Surgery, Oncology and Gastroenterology, University of Padua, Via Giustiniani 2, 35128, Padua, Italy.
    Endocrine tumors of thyroid, adrenal and parathyroid glands may be due to germline and inheritable mutations in 5-30% of patients. Medullary Thyroid Carcinoma, Pheochromocytoma, Paraganglioma, and Familial Primary Hyperparathyroidism are the most frequent entity. Hereditary endocrine tumors usually have a suggestive familial history; they occur earlier than sporadic variants, are multifocal, and have increased recurrence rates. Read More

    Malignant pheochromocytoma in the anterior mediastinum with sternal invasion: a case report.
    J Thorac Dis 2017 Mar;9(3):E202-E209
    Department of Thoracic Surgery, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, China.
    Pheochromocytomas are catecholamine-producing neuroendocrine tumors that usually occur in the adrenal medulla or sympathetic paraganglia. Anterior mediastinum involvement with pheochromocytoma is rare and may not present with typical symptoms. Its clinical manifestation may be unclear and a high index of suspicion is required for accurate diagnosis. Read More

    Adrenal Vein Catecholamine Levels and Ratios: Reference Intervals Derived from Patients with Primary Aldosteronism.
    Horm Metab Res 2017 Apr 26. Epub 2017 Apr 26.
    Department of Endocrinology, St Bartholomew's Hospital, London, UK.
    Phaeochromocytoma localisation is generally reliably achieved with modern imaging techniques, particularly in sporadic cases. On occasion, however, there can be diagnostic doubt due to the presence of bilateral adrenal abnormalities, particularly in patients with mutations in genes predisposing them to the development of multiple phaeochromocytomas. In such cases, surgical intervention is ideally limited to large or functional lesions due to the long-term consequences associated with hypoadrenalism. Read More

    Incidence and prevalence of multiple endocrine neoplasia 2B in Denmark: a nationwide study.
    Endocr Relat Cancer 2017 Apr 24. Epub 2017 Apr 24.
    C Godballe, Department of ORL Head & Neck Surgery, Odense University Hospital, Odense, Denmark.
    Extract: Multiple endocrine neoplasia 2B (MEN2B) is an autosomal dominant inherited cancer syndrome associating medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), ganglioneuromatosis of the aerodigestive tract and facial, ophthalmologic and skeletal abnormalities. MEN2B is caused by the M918T and A883F mutation of the REarranged during Transfection (RET) proto-oncogene in approximately 95% and <5% of cases, respectively. Only very few other mutations have been reported to cause MEN2B. Read More

    Cushing Disease in a patient with Multiple Endocrine Neoplasia type 2B.
    J Clin Transl Endocrinol Case Rep 2017 Jun;4:1-4
    National Institute of Child Health & Human Development, National Institutes of Health (NIH), Building 10-CRC, room 1-3330 10 Center Drive Bethesda MD 20892.
    Context: Multiple endocrine neoplasia type 2B (MEN2B) is a rare autosomal-dominant cancer syndrome characterized in part by metastatic medullary thyroid cancer (MTC) and pheochromocytoma. Cushing disease is a rare cause of endogenous hypercortisolism in children.

    Case Description: We describe a 21-year-old African-American male who was diagnosed at age 10 with an ACTH-secreting pituitary microadenoma. Read More

    Genotype-phenotype correlation in paediatric pheochromocytoma and paraganglioma: a single centre experience from India.
    J Pediatr Endocrinol Metab 2017 Apr 22. Epub 2017 Apr 22.
    Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai.
    Background: Data on genotype-phenotype correlation in children is limited. Hence, we studied the prevalence of germline mutations and genotype-phenotype correlation in children with pheochromocytoma (PCC)/paraganglioma (PGL) and compared it with adult PCC/PGL cohort.

    Methods: A total of 121 consecutive, unrelated, index PCC/PGL patients underwent genetic testing for five PCC/PGL susceptibility genes (RET, VHL, SDHB, SDHD and SDHC) and were evaluated for clinical diagnosis of neurofibromatosis type1 (NF1). Read More

    Raynaud's Phenomenon: Revisiting a Rare Sign of Pheochromocytoma/Paraganglioma.
    Urology 2017 Apr 18. Epub 2017 Apr 18.
    Paraganglioma (PGL) are rare tumors arising from extraadrenal chromaffin cells and occasionally secret catecholamines. The patient commonly presents with headache, palpitation, anxiety, diaphoresis and episodic or sustained hypertension. Rarely patient can present with Raynaud's phenomenon. Read More

    Propranolol.
    Profiles Drug Subst Excip Relat Methodol 2017 6;42:287-338. Epub 2017 Apr 6.
    College of Pharmacy, King Saud University, Riyadh, Saudi Arabia.
    Propranolol is a noncardioselective β-blocker. It is reported to have membrane-stabilizing properties, but it does not own intrinsic sympathomimetic activity. Propranolol hydrochloride is used to control hypertension, pheochromocytoma, myocardial infarction, cardiac arrhythmias, angina pectoris, and hypertrophic cardiomyopathy. Read More

    Metabolic regulation and the anti-obesity perspectives of human brown fat.
    Redox Biol 2017 Apr 11;12:770-775. Epub 2017 Apr 11.
    The Centre of Inflammation and Metabolism and Centre for Physical Activity Research Rigshospitalet, University Hospital of Copenhagen, Denmark.
    Activation of brown adipose tissue (BAT) in adult humans increase glucose and fatty acid clearance as well as resting metabolic rate, whereas a prolonged elevation of BAT activity improves insulin sensitivity. However, substantial reductions in body weight following BAT activation has not yet been shown in humans. This observation raise the possibility for feedback mechanisms in adult humans in terms of a brown fat-brain crosstalk, possibly mediated by batokines, factors produced by and secreted from brown fat. Read More

    Establishment and evaluation of a novel biomarker-based nomogram for malignant phaeochromocytomas and paragangliomas.
    Clin Endocrinol (Oxf) 2017 Apr 21. Epub 2017 Apr 21.
    Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Key Laboratory for Endocrine and Metabolic Diseases of Chinese Health Ministry, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, 197 Ruijin 2nd Road, Shanghai, 200025, P.R. China.
    Objective: No single histological or molecular marker is diagnostic for malignant phaeochromocytomas and paragangliomas (PPGLs). This study aimed to establish and evaluate a prognostic nomogram to improve the prediction of metastatic probability in individual PPGL patients.

    Methods: Three hundred and forty-seven consecutive PPGL patients from January 2002 through December 2014 were randomly divided into a training set (n=208) and a validation set (n=139). Read More

    Ultrastructure of pheochromocytoma: undescribed morphologic features.
    Virchows Arch 2017 Apr 20. Epub 2017 Apr 20.
    Department of Pathology, Clinical University Hospital, Galician Health Service (SERGAS), Health Research Institute of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.
    We examined samples of human pheochromocytoma from 11 patients aged 30-70 years including one case of malignant pheochromocytoma with a view to identifying previously unreported ultrastructural details.We identified two types of nuclear inclusions consisting of irregularly shaped singular or multiple granulofibrillar formations with a typical concentric halo, on the one hand, and accumulations of egg-shaped structures consisting of granules and microfilaments, on the other. In some of the tumor cells, membrane-covered inclusions containing parallel laminar elements arranged in a paracrystalline, periodic fashion, or mega-mitrochondriae characterized by increased electrodensity of their matrix, and fibrillary material in the spaces between the cristae were present. Read More

    Anthracyclines suppress pheochromocytoma cell characteristics, including metastasis, through inhibition of the hypoxia signaling pathway.
    Oncotarget 2017 Apr;8(14):22313-22324
    Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.
    Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare, neuroendocrine tumors derived from adrenal or extra-adrenal chromaffin cells, respectively. Metastases are discovered in 3-36% of patients at the time of diagnosis. Currently, only suboptimal treatment options exist. Read More

    Adrenalectomy: indications and options for treatment.
    Updates Surg 2017 Apr 18. Epub 2017 Apr 18.
    General, Emergency and Minimally Invasive Surgery Unit, Careggi University Hospital, Largo Brambilla 3, 50134, Florence, Italy.
    The history of adrenal surgery is longstanding. Firstly described in 1889 by Thornton, the open adrenalectomy has been for decades the only surgical approach to adrenal diseases. Nowadays, instead, several approaches to adrenal glands have been described in the literature, such as laparoscopic adrenalectomy, robotic-assisted procedure and single-incision technique. Read More

    Pheochromocytoma of the Organ Zuckerkandl.
    J La State Med Soc 2017 Mar-Apr;169(2):53. Epub 2017 Apr 15.
    Department of Internal Medicine, LSU Health Sciences Center, New Orleans, LA.
    Introduction: Pheochromocytomas (PCCs);, or intra-adrenal paragangliomas (PGLs);, are neuroendocrine tumors arising within the adrenal medulla. Extra-adrenal paragangliomas may arise in the sympathetic or parasympathetic paraganglia and more rarely in other organs. One of the most common extra-adrenal sites is in the organ of Zuckerkandl, a collection of chromaffin cells near the origin of the inferior mesenteric artery or near the aortic bifurcation. Read More

    Comparative evaluation of iodine-131 metaiodobenzylguanidine and 18-fluorodeoxyglucose positron emission tomography in assessing neural crest tumors: Will they play a complementary role?
    South Asian J Cancer 2017 Jan-Mar;6(1):31-34
    Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre, Mumbai, Maharashtra, India.
    Background: 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) has established a role in the evaluation of several malignancies. However, its precise clinical role in the neural crest cell tumors continues to evolve.

    Purpose: The purpose of this study was to compare iodine-131 metaiodobenzylguanidine ((131)I-MIBG) and FDG-PET of head to head in patients with neural crest tumors both qualitatively and semiquantitatively and to determine their clinical utility in disease status evaluation and further management. Read More

    Paraganglioma of the bladder in a kidney transplant recipient: A case report.
    Mol Clin Oncol 2017 Apr 6;6(4):553-555. Epub 2017 Mar 6.
    Department of Nephrology and Kidney Transplantation, Necker Enfants-Malades Hospital, Paris Descartes University, Assistance Publique-Hôpitaux de Paris, 75015 Paris, France.
    Renal transplantation has been associated with a significantly increased risk of developing cancer, including bladder neoplasia, with urothelial carcinoma being the most frequent type of bladder cancer. Bladder paraganglioma, also referred to as extra-adrenal pheochromocytoma, is a rare but severe condition that may cause a severe hypertensive crisis during handling and mobilization of the tumor. We herein present the case of a 67-year-old kidney transplant recipient with a bladder polyp consistent with paraganglioma of the bladder. Read More

    microRNA 183 family profiles in pheochromocytomas are related to clinical parameters and SDHB expression.
    Hum Pathol 2017 Apr 12. Epub 2017 Apr 12.
    Cancer Molecular Pathology, School of Medicine and Menzies Health Institute Queensland, Griffith University, Gold Coast, Australia. Electronic address:
    This study aims to examine the expression profiles of the miR-183 cluster (miR-96/182/183) in pheochromocytoma. Pheochromocytoma tissues were prospectively collected from 50 patients with pheochromocytoma. Expression of miR-183 cluster members and SDHB protein expression were analysed in these tissues by quantitative real-time polymerase chain reaction and immunohistochemistry respectively. Read More

    Failure of MIBG scan to detect metastases in SDHB-mutated pediatric metastatic pheochromocytoma.
    Pediatr Blood Cancer 2017 Apr 14. Epub 2017 Apr 14.
    Department of Pediatrics, Memorial Sloan Kettering Cancer Centre, New York, New York.
    (123) I-meta-iodo benzyl guanidine (MIBG) scans are considered the gold standard imaging in neuroblastoma; however, flouro deoxy glucose positron emission tomography (FDG-PET) scans have increased sensitivity in adults with pheochromocytoma/paraganglioma. We describe a pediatric patient initially considered to have localized neuroblastoma based on anatomical imaging and (123) I-MIBG scan, but subsequent investigations revealed germline succinate dehydrogenase complex iron sulfur subunit B (SDHB) mutation-associated pheochromocytoma with multiple FDG-avid skeletal metastases. We then compared (123) I-MIBG and FDG-PET scans in children with metastatic pheochromocytoma/paraganglioma. Read More

    Cerebrosides from Sea Cucumber Protect Against Oxidative Stress in SAMP8 Mice and PC12 Cells.
    J Med Food 2017 Apr;20(4):392-402
    1 College of Food Science and Engineering, Ocean University of China , Qingdao, China .
    Alzheimer's disease (AD) is a neurodegenerative disorder. Emerging evidence implicates β-amyloid (Aβ) plays a critical role in the progression of AD. In this study, we investigated the protective effect of cerebrosides obtained from sea cucumber against senescence-accelerated mouse prone 8 (SAMP8) mice in vivo. Read More

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