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pheochromocytoma guidelines

Anju Sahdev
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 summarises and highlights radiological recommendations within the recently issued guidelines for the management of adrenal incidentalomas from European Society of Endocrinology Clinical Practice in collaboration with European Network for Study of Adrenal Tumours...
February 9, 2017: British Journal of Radiology
Shunsaku Takayanagi, Akitake Mukasa, Hirofumi Nakatomi, Hiroshi Kanno, Jun-Ichi Kuratsu, Ryo Nishikawa, Kazuhiko Mishima, Atushi Natsume, Toshihiko Wakabayashi, Kiyohiro Houkin, Shunsuke Terasaka, Masahiro Yao, Nobuo Shinohara, Taro Shuin, Nobuhito Saito
von Hippel-Lindau (VHL) disease is a hereditary tumor disease in which tumors develop in multiple organs, not only as hemangioblastomas (HBs) in the central nervous system, but also as kidney tumors, pheochromocytomas, and so on. Much about the epidemiology of VHL disease remained unknown until fairly recently in Japan, leading to calls for the establishment of a VHL disease epidemiological database in Japanese. To elucidate its epidemiology in Japan, the Japanese Ministry of Health, Labour and Welfare created the VHL Disease Study Group, which was put in charge of carrying out a nationwide epidemiological survey...
January 6, 2017: Neurologia Medico-chirurgica
Elisabeth Joye Petr, Tobias Else
Endocrine tumor syndromes, eg, multiple endocrine neoplasia types 1 and 2, were among the first recognized hereditary predisposition syndromes to tumor development. Over time, the number of endocrine tumor syndromes has significantly expanded, eg, with the recent inclusion of hereditary paraganglioma syndromes. Associations of non-endocrine tumors with hereditary endocrine tumor syndromes and endocrine tumors with non-classical endocrine tumor syndromes have emerged. These findings have certainly expanded the scope of care, necessitating a multidisciplinary approach by a team of medical professionals and researchers, integrating shared patient decision-making at every step of surveillance, diagnosis, and treatment...
October 2016: Seminars in Oncology
Henrique V Luiz, Run Yu, Katherine Wolf, Ning Miao, Andrew Mannes, Karel Pacak
No abstract text is available yet for this article.
December 2016: Clinical Endocrinology
Martin Fassnacht, Wiebke Arlt, Irina Bancos, Henning Dralle, John Newell-Price, Anju Sahdev, Antoine Tabarin, Massimo Terzolo, Stylianos Tsagarakis, Olaf M Dekkers
: By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system...
August 2016: European Journal of Endocrinology
Rachel Wilson, Nausheen Syed, Prabodh Shah
The association of mutations in the PHD2 protein of the hypoxia-sensing pathway and erythrocytosis has only been established in the last decade. Here we report the case of a novel PHD2 gene mutation in a patient with erythrocytosis and summarize all reported cases to date. Case Report. A 55-year-old man presented with dyspnea and a previous diagnosis of idiopathic erythrocytosis. PHD gene sequencing revealed a mutation on exon 2. The mutation was recognized as p.(Trp334(⁎)) (c. 1001G>A) resulting in a truncation of a highly conserved amino acid residue in catalytic domain...
2016: Case Reports in Hematology
Ingo Janssen, Clara C Chen, Corina M Millo, Alexander Ling, David Taieb, Frank I Lin, Karen T Adams, Katherine I Wolf, Peter Herscovitch, Antonio T Fojo, Inga Buchmann, Electron Kebebew, Karel Pacak
PURPOSE: Pheochromocytomas/paragangliomas (PPGLs) and their metastases are tumors that predominantly express somatostatin receptor 2 (SSR2). (68)Ga-DOTA(0)-Tyr(3)-octreotate ((68)Ga-DOTATATE) is a PET radiopharmaceutical with both high and selective affinity for SSRs. The purpose of this study was to evaluate the utility of (68)Ga-DOTATATE in comparison with other specific and nonspecific radiopharmaceuticals recommended in the current guidelines for the localization of metastatic sporadic PPGL by PET/CT...
September 2016: European Journal of Nuclear Medicine and Molecular Imaging
Attila Patócs, István Likó, Henriett Butz, Kornélia Baghy, Károly Rácz
The technical developments leading to revolution in clinical genetic testing offer new approaches for patients with cancer. From one mutation or one gene approach the scale of genetic testing moved to whole exome or whole genome scale. It is well known that many tumours are genetically determined and they are part of familial tumour syndromes. In addition, some mutations indicate specific molecular targeted therapies. Although sampling and sample preparation are different for testing germline and somatic mutations, the technical background of the analysis is the same...
December 20, 2015: Orvosi Hetilap
Seigo Kinuya, Keiichiro Yoshinaga, Tetsuya Higuchi, Megumi Jinguji, Hiroshi Kawamoto, Hiroaki Kurihara
131I-MIBG radiotherapy has been used for unresectable nueroendocrine tumors including malignant pheochromocytomas and neuroblastomas in foreign countries since the '80s when clinical therapeutic trials were initiated. In Japan, 131I-MIBG radiotherapy has not been approved by Ministry of Health, Labor and Welfare, however, personally imported 131I-MIBG is now available in limited institutions for therapeutic purpose. This updated guideline draft aims to provide useful information concerning 131I-MIBG radiotherapy, to prevent side effects, and to protect physicians, nurses, other health care professionals, patients and their families from radiation exposure...
February 2015: Kaku Igaku. the Japanese Journal of Nuclear Medicine
Rachel D Aufforth, Pooja Ramakant, Samira M Sadowski, Amit Mehta, Katarzyna Trebska-McGowan, Naris Nilubol, Karel Pacak, Electron Kebebew
CONTEXT: Patients with von Hippel-Lindau (VHL) syndrome have a 25-30% chance of developing pheochromocytoma. Although practice guidelines recommend biochemical and radiological screening every 1-2 years for pheochromocytoma in patients with VHL, there are limited data on the optimal age and frequency for screening. OBJECTIVE: Our objective was to determine the earliest age of onset and frequency of contralateral and recurrent pheochromocytomas in patients with VHL syndrome...
December 2015: Journal of Clinical Endocrinology and Metabolism
Cassandre E Bénay, Mehdi Tahiri, Lawrence Lee, Evangelia Theodosopoulos, Amin Madani, Liane S Feldman, Elliot J Mitmaker
BACKGROUND: Guidelines recommend 24-48 hours of intensive monitoring after resection of pheochromocytoma. However, many patients do not require it. The objective of this study is to identify preoperative risk factors associated with postoperative hemodynamic instability (HDI) so as to select patients who may not require intensive postoperative monitoring. METHODS: Medical records of patients undergoing pheochromocytoma resection over a 12-year period were reviewed...
January 2016: Surgery
Camilla Schalin-Jäntti, Merja Raade, Esa Hämäläinen, Timo Sane
BACKGROUND: Current guidelines for follow-up of adrenal incidentalomas are extensive and hampered by lack of follow-up studies. We tested the hypothesis that small lipid-rich adrenal incidentalomas, initially characterized by tumor size <40 mm and <10 Hounsfield units (HUs) on unenhanced computed tomography (CT) may not demonstrate excessive growth/hormonal hypersecretion on follow-up. METHODS: Sixty-nine incidentalomas in 56 patients were restudied with unenhanced CT and screening for hypercortisolism (dexamethasone suppression test [DST], plasma adrenocorticotropic hormone) and pheochromocytoma (24-hour urinary metanephrines and normetanephrines) 5 years later...
December 2015: Endocrinology and Metabolism
Anne-Paule Gimenez-Roqueplo
Pheochromocytoma and functional paraganglioma (PH/PGL) are classical causes of secondary hypertension. The clinical practice guidelines for PH/PGL have been changed by the recent identification of a dozen of susceptibility genes. PH/PGL is the neuroendocrine tumor most affected by genetics. Total metanephrin should be measured in every young patient suffering of a resistant hypertension. The diagnosis is based on conventional imaging assciated with nuclear imaging. Genetic testing should be offered to every patient diagnosed for PH/PGL because the identification of a germline mutation, which is found in over 30% of the cases, will change his work-up and follow-up as well as offer the opportunity of a familial genetic testing in relatives...
June 2015: La Revue du Praticien
T Zelinka, O Petrák, B Hamplová, H Turková, P Waldauf, J Rosa, Z Šomlóová, R Holaj, B Štrauch, T Indra, M Kršek, A Brabcová Vránková, A Brabcová Vránková, Z Musil, J Dušková, J Kubinyi, D Michalský, K Novák, J Widimský
Pheochromocytomas are catecholamine-producing tumors with typical clinical presentation. Tumor resection is considered as an appropriate treatment strategy. Due to its unpredictable clinical behavior, biochemical testing is mandatory to confirm the success of tumor removal after surgery. The aim of the study was to investigate the feasibility of a shorter interval of postoperative testing (earlier than the recommended 2-4 weeks according to recently published Guidelines). We investigated 81 patients with pheochromocytoma before and after surgery...
August 2015: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
Jekaterina Patrova, Iwona Jarocka, Hans Wahrenberg, Henrik Falhammar
OBJECTIVE: To investigate the outcome in patients with adrenal incidentaloma (AI). METHODS: A retrospective evaluation of 637 patients with AI referred to a tertiary center over 8 years. Radiologic and hormonal evaluations were performed at baseline. Follow-up imaging was carried out if necessary, and hormonal evaluation was performed at 24 months according to national guidelines. RESULTS: The mean age was 62.7 ± 11.6 years, and the mean AI size was 25...
August 2015: Endocrine Practice
Darko Kastelan, Ivana Kraljevic, Tina Dusek, Nikola Knezevic, Mirsala Solak, Bojana Gardijan, Marko Kralik, Tamara Poljicanin, Tanja Skoric-Polovina, Zeljko Kastelan
OBJECTIVE: The current guidelines for the management of adrenal incidentaloma advise hormonal and radiological follow-up of patients for 2-5 years after the initial diagnosis. However, the vast majority of adrenal incidentaloma are non-functional benign cortical adenomas that require no treatment, so the routine application of the current strategies often results in a number of unnecessary biochemical and radiological investigations. The aim of this study was to analyse the clinical course of patients with adrenal incidentaloma and to provide a critical review of the current management strategy of the disease...
August 2015: European Journal of Endocrinology
Ioannis Ilias, Georgios Meristoudis, Athanasios Notopoulos
UNLABELLED: Paragangliomas (PGL) and pheochromocytomas (P) are rare neural-crest-derived neoplasms. Very recently guidelines on diagnosis and treatment of PGL/P have been presented by the US Endocrine Society. In the following overview we assessed the implementation of these guidelines with probabilistic reasoning (calculating with Fagan nomograms the post-test probability of PGL/P for a given pre-test probability). CONCLUSION: Biochemical evaluation of PGL/P showed excellent diagnostic characteristics with post-test probabilities that are very different from the pre-test probabilities, thus a positive biochemical test is usually indicative of disease whereas a negative one usually rules out disease...
January 2015: Hellenic Journal of Nuclear Medicine
Konstantinos Kiroplastis, Apostolos Kambaroudis, Apostolos Andronikou, Andromachi Reklou, Dimitris Kokkonis, Panagiotis Petras, Apostolos Mamopoulos, Eudokia Anagnostara, Charalampos Spyridis
Purpose. Pheochromocytoma in association with pregnancy is a very rare, without specific symptoms, life-threatening condition, increasing both maternal and fetal mortality up to 50%. The present paper illustrates the case of a pregnant woman, diagnosed with pheochromocytoma, aiming to demonstrate and discuss the difficulties that arouse during the diagnosis and the problems concerning the treatment. Patient. A 34-year-old woman, in the 9th week of pregnancy, complained for headache, sweating, and a feeling of heavy weight on the right renal area...
2015: Case Reports in Obstetrics and Gynecology
Seigo Kinuya, Keiichiro Yoshinaga, Tetsuya Higuchi, Megumi Jinguji, Hiroaki Kurihara, Hiroshi Kawamoto
Since the 1980s when clinical therapeutic trials were initiated, (131)I-MIBG radiotherapy has been used in foreign countries for unresectable neuroendocrine tumors including malignant pheochromocytomas and neuroblastomas. In Japan, (131)I-MIBG radiotherapy has not been approved by the Ministry of Health, Labour and Welfare; however, personally imported (131)I-MIBG is now available for therapeutic purposes in a limited number of institutions. These updated draft guidelines aim to provide useful information concerning (131)I-MIBG radiotherapy, to help prevent side effects and protect physicians, nurses, other health care professionals, patients and their families from radiation exposure...
July 2015: Annals of Nuclear Medicine
Kirk J Pak, Tian Hu, Colin Fee, Richard Wang, Morgan Smith, Lydia A Bazzano
BACKGROUND: Few clinical practice guidelines provide management recommendations for acute hypertensive episodes except in the context of specific conditions such as pregnancy and stroke. METHODS: We performed a systematic search to identify guidelines addressing acute hypertension and appraised the guidelines using the Appraisal of Guidelines for Research and Evaluation (AGREE II) validated quality assessment tool. Two reviewers independently appraised and one extracted key recommendations...
2014: Ochsner Journal
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