Pheochromocytoma and paraganglioma (PPGLs) are rare chromaffin cell tumors with a variable prevalence depending on the investigated population. [Medline]. Parikh PP, Rubio GA, Farra JC, Lew JI. 2003 Sep. 21(9):1703-7. 2017 Jul. [37] : For imaging studies, the joint ES/AACC/ESE guidelines recommend CT scanning as first line, rather than MRI. 2018 Feb 21. 2018 Jan 17. pheochromocytoma (PGL/PCC) syndrome when all of the following criteria are met: • Individual meets general criteria for hereditary cancer genetic testing (above) • Individual with pheochromocytoma or paraganglioma • Other syndromes and causes of PGL/PCC … J Clin Endocrinol Metab. 1991 Jun. Exp Brain Res. Clin Endocrinol (Oxf). 2017 Aug 10. AJR Am J Roentgenol. La Batide-Alanore A, Chatellier G, Plouin PF. Baid SK, Lai EW, Wesley RA, Ling A, Timmers HJ, Adams KT, et al. 11C-hydroxyephedrine positron emission tomography imaging of pheochromocytoma: a single center experience over 11 years. A rare, hereditary, pheochromocytoma/paraganglioma tumor arising from neuroendocrine chromaffin cells of the adrenal medulla (pheochromocytoma) or from any paraganglia from the skull base to the pelvic floor (paraganglioma). Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma. Tucker ME. 161(3):157-60. 2021 Mar 11;8:648725. doi: 10.3389/fcvm.2021.648725. Perioperative hemodynamics and outcomes of patients on metyrosine undergoing resection of pheochromocytoma or paraganglioma. [Medline]. 88(6):2656-66. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0MDU5LWd1aWRlbGluZXM=, Patients who have a hereditary risk for developing a pheochromocytoma or paraganglioma (extra-adrenal pheochromocytoma), Plasma free or 24-hour urine fractionated metanephrines, Contrast-enhanced chest/abdominal/pelvic multiphasic CT scanning or MRI, Patients with PPGLs should be engaged in shared decision-making for genetic testing, Patients with paraganglioma should undergo testing of succinate dehydrogenase mutations, Patients with metastatic disease should undergo testing for, Genetic testing should include pretest and posttest genetic counseling, Blockade of hormonally functional PPGL to prevent cardiovascular complications, Medical treatment to normalize blood pressure and heart rate, A high-sodium diet with supplemental fluid intake to prevent severe hypotension after removal of the tumor, Surgical debulking to release tumor pressure on surrounding tissues or to decrease tumor mass, In select patients, radiofrequency ablation or cryoablation are options, Chemotherapy is preferred in patients with negative MIBG scintigraphy and in those with rapidly progressing tumors, External-beam irradiation of bone metastases or radiofrequency and cryoablation in selected cases only. Endocrine Society first to address rare adrenal-tumor care. Pravenec M, Kren V, Klír P, Simonet L, Kurtz T. Identification of genes determining spontaneous hypertension. Iglesias P, Santacruz E, Garcia-Sancho P, et al. Sheps SG, Jiang NS, Klee GG, van Heerden JA. GUIDELINES MADE SIMPLE 2017 Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults BP 5 Back to Table of Contents Of˜ce BP: ≥130/80 mm Hg but <160/100 mm Hg after 3 mo trial of lifestyle modi˚cation and suspect white coat hypertension Yes No Daytime ABPM or HBPM BP <130/80 mm Hg White Coat Hypertension Confirmed: Diet Influences Colorectal Cancer Risk, CRC Screening to Start at Age 45: ACG Update. The Task Force followed the approach recommended … [Medline]. 2017 Jul 13. Front Cardiovasc Med. Prevention and treatment information (HHS). The 2010 NANETS recommendations for treatment of advanced disease include the following eCollection 2020. 5:155. FOIA 57(3):411-20. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Catecholamine metabolomic and secretory phenotypes in phaeochromocytoma. 2007 Apr. The name is of Greek etymology. Epub 2011 Sep 2. 6-18F-fluoro-L-dihydroxyphenylalanine positron emission tomography is superior to 123I-metaiodobenzyl-guanidine scintigraphy in the detection of extraadrenal and hereditary pheochromocytomas and paragangliomas: correlation with vesicular monoamine transporter expression. May 25, 2016; Accessed: October 2, 2016. Pheochromocytoma and pregnancy: a deceptive connection.Eur J Endocrinol. [Medline]. 30(11):1031-62. J Clin Endocrinol Metab. Exp Clin Endocrinol Diabetes. gallium-ga-68-dotatoc-40000
[66] : For preoperative management, ES/AACC/ESE recommendations include the following Lenders JW, Duh QY, Eisenhofer G, et al; Endocrine Society. 2010 Jan. 95(1):238-45. Eur J Pediatr. The 2010 guidelines from the North American Neuroendocrine Tumor Society (NANETS) recommend biochemical testing for pheochromocytoma that includes measurements of fractionated metanephrines in plasma, urine, or both, as available, in the following cases SIGNS & SYMPTOMS: Symptoms occur in ~50% of patients and are often episodic and paroxysmal in nature. Fottner C, Helisch A, Anlauf M, Rossmann H, Musholt TJ, Kreft A, et al. [Medline]. 2010 May. Low sensitivity of glucagon provocative testing for diagnosis of pheochromocytoma. Usefulness of 123I-MIBG scintigraphy in the evaluation of patients with known or suspected primary or metastatic pheochromocytoma or paraganglioma: results from a prospective multicenter trial. Donatini G, Kraimps JL, Caillard C, Mirallie E, Pierre F, De Calan L, Hamy A, Larin O, Tovkay O, Cherenko S. Surg Endosc. J Clin Endocrinol Metab. 2009 Feb. 192(2):450-4. Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. [66]. 2007 Nov. 17(11):2804-9. Last published: 2016. J Hypertens. [67] : For genetic testing, ES/AACC/ESE recommendations are as follows [Medline]. [Medline]. J Clin Endocrinol Metab. Drugs, 2010
A pheochromocytoma results in the irregular and excessive release of these hormones. Clinical value of 18F-fluorodihydroxyphenylalanine positron emission tomography/computed tomography (18F-DOPA PET/CT) for detecting pheochromocytoma. [Medline]. [Medline]. Review of Pediatric Pheochromocytoma and Paraganglioma. N Engl J Med. Drug Saf. Blake MA, Kalra MK, Maher MM, Sahani DV, Sweeney AT, Mueller PR, et al. Eur J Intern Med. eCollection 2021. Phios means dusky, chroma means colour and cytoma means tumour. Pheochromocytoma diagnosed during pregnancy: lessons learned from a series of ten patients. Endocrine Society (ENDO) Annual Meeting. Ann Surg. Careers. [67] : If metastatic disease is suspected, the guidelines recommend the following additional tests The patient needs pre-treatment with α1-blockers at least 10-14 days before operation. [Medline]. Kim S, Salibi N, Hardie AD, Xu J, Lim RP, Lee VS, et al. 2017 Jul 13. . Unilateral subtotal adrenalectomy for pheochromocytoma in multiple endocrine neoplasia type 2 patients: a feasible surgical strategy. [Medline]. Eisenhofer G, Goldstein DS, Walther MM, Friberg P, Lenders JW, Keiser HR, et al. Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. /viewarticle/941888
46:1-6. 99(6):1915-42. 2009 Sep. 161(3):495-502. Kwon SY, Lee KS, Lee JN, et al. Review: Should patients with apparently sporadic pheochromocytomas or paragangliomas be screened for hereditary syndromes?. INTRODUCTION. [Medline]. Reversible cerebral vasoconstriction syndrome (RCVS): an interesting case report. Non-neoplastic adrenal cortex (yellow) surrounds a small tan-red tumor in the medullary region, representing a pheochromocytoma. Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. The 5th International Symposium on Pheochromocytoma and Paraganglioma will be held at the Sofitel Sydney Wentworth, Australia from August 30 to September 2, 2017. Azedra (iobenguane I 131) in patients with metastatic and/or recurrent and/or unresectable pheochromocytoma or paraganglioma: biochemical tumor marker results of a multicenter, open-label pivotal phase 2 study (OR02-5). 2014 Jun;99(6):1915-42. doi: 10.1210/jc.2014-1498. [Medline]. Eur Radiol. Thompson LD. administration of nonionic contrast material. 2018 Sep;32(9):3890-3900. doi: 10.1007/s00464-018-6128-x. Quick MRI Scan for Routine Prostate Cancer Screening? Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. This can lead to high blood pressure and cause symptoms such as severe headaches, irritability, sweating, rapid heart rate, nausea, vomiting, weight loss, weakness, chest pain, and anxiety. Surgery. We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO). H and E, high power, showing classic "balls of cells" feature of a pheochromocytoma. Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr; Endocrine Society. Clinical Predictors of Malignancy in Patients with Pheochromocytoma and Paraganglioma. Pheochromocytoma Is Characterized by Catecholamine-Mediated Myocarditis, Focal and Diffuse Myocardial Fibrosis, and Myocardial Dysfunction. Keywords: Michael A Blake, MBBCh, MRCPI, FRCR is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, Royal College of Physicians of Ireland, Royal College of Surgeons in IrelandDisclosure: Received royalty from Springer for book editor. A total of 68 procedures for pheochromocytoma were performed between June 1997 and february 2017. Re-evaluation of pheochromocytomas on delayed contrast-enhanced CT: washout enhancement and other imaging features. JAMA. 2014 Jun. 2010 Jun. N Engl J Med. Robotic adrenalectomy in patients with pheochromocytoma: a systematic review. Barone JE. Eng C. Seminars in medicine of the Beth Israel Hospital, Boston. DEsIgn: This study is a retrospective review. Mayo Clin Proc. Clin Chem. 13(3):269-73. 2010 Jun. [Medline]. Postoperative measurement of plasma or urine metanephrine levels and lifelong annual biochemical testing are suggested. All material on this website is protected by copyright, Copyright © 1994-2021 by WebMD LLC. Pheochromocytoma. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. The use of liquid chromatography with mass spectrometry or electrochemical detection methods is suggested over other laboratory methods. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing. The guidelines include recommendations (based on strong evidence) and suggestions (based on weaker evidence). June 5, 2014. 2012 May 15. Several familial syndromes are associated with PHEO and genetic testing should be considered. 2021 Apr 6;22(1):20. doi: 10.1186/s10194-021-01225-7. [66]. 95(5):2023-37. Endocr Pract. Pheochromocytoma Crisis Is Not a Surgical Emergency. J Clin Endocrinol Metab. Open resection is suggested for paragangliomas, although laparoscopic resection is an option for smaller tumors. Accessed: January 29, 2013. 2017 Jul 26. 2002 Mar 20. [66]. Leading worldwide experts in the field will gather to present their latest data, share clinical conundrums, showcase new therapeutic approaches, and develop strong international partnerships. http://www.medscape.com/viewarticle/777889, American Association of Clinical Endocrinologists, American Association for the Advancement of Science, International Society for Clinical Densitometry, Southern Society for Clinical Investigation, American College of Medical Practice Executives, American Association for Physician Leadership, Central Society for Clinical and Translational Research. Epub 2018 Feb 27. 2016 May. de Jong WH, Eisenhofer G, Post WJ, Muskiet FA, de Vries EG, Kema IP. The National Comprehensive Cancer Network guidelines recommend the following tests for the evaluation of suspected PPGL Eur J Endocrinol. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Am J Med Genet. Hamidi O, Young WF Jr, Gruber L, et al. 165(1):91-6. Characterization of adrenal pheochromocytoma using respiratory-triggered proton MR spectroscopy: initial experience. [Medline]. J Clin Oncol. Boulkina LS, Newton CA, Drake AJ 3rd, Tanenberg RJ. Butz JJ, Weingarten TN, Cavalcante AN, et al. Luster M, Karges W, Zeich K, Pauls S, Verburg FA, Dralle H, et al. J Nucl Med. J Clin Endocrinol Metab. 2017 Sep 14. ACCURACY OF PLASMA FREE METANEPHRINES IN THE DIAGNOSIS OF PHEOCHROMOCYTOMA AND PARAGANGLIOMA: A SYSTEMATIC REVIEW AND META-ANALYSIS. Discover the world's research 20+ million members Its prevalence in full-term pregnancies is reported to be 1/50,000 [ 1, 4, 6 ]. Scholten A, Cisco RM, Vriens MR, Cohen JK, Mitmaker EJ, Liu C, et al. The mass is a pheochromocytoma. Butz JJ, Yan Q, McKenzie TJ, et al. The American Joint Committee on Cancer staging system for pheochromocytomas and sympathetic paragangliomas was implemented in 2017 to guide clinicians in determining the therapies and follow-up that patients require . SDHAF2 (PGL2-SDH5) and hereditary head and neck paraganglioma. [Medline]. Axial, T2-weighted magnetic resonance imaging (MRI) scan showing large left suprarenal mass of high signal intensity on a T2-weighted image. Ther Adv in Endo and Metab. Coexistence of osteoporosis and atherosclerosis in pheochromocytoma: new insights into its long-term management. 2010 Mar. January 18, 2013. [Medline]. Overall, benign, non-functioning adrenal adenomas account for about 80% of adrenal incidentalomas. 50(3):306-10. [Medline]. Scholten A, Valk GD, Ulfman D, Borel Rinkes IH, Vriens MR. N Engl J Med. [Medline]. [Full Text]. [Medline]. Szolar DH, Korobkin M, Reittner P, Berghold A, et al. Paladino NC, Lowery A, Guérin C, Taïeb D, Sebag F. Ann Ital Chir. 215:204-10. [Medline]. [Full Text]. Arnault-Ouary G, Chatal JF, Charbonnel B. Porcaro AB, Novella G, Ficarra V, D'Amico A, Antoniolli SZ, Curti P. Arch Ital Urol Androl. Pheochromocytoma crisis is not a surgical emergency. Li QY, Li F. Laparoscopic adrenalectomy in pheochromocytoma: retroperitoneal approach versus transperitoneal approach. Yamamoto S, Hellman P, Wassberg C, Sundin A. Accessibility If you log out, you will be required to enter your username and password the next time you visit. Mayo Clin Proc. They belong to a group of diseases termed neuroendocrine tumors (NETs). Review article. 2017 Sep 7. Jacobson AF, Deng H, Lombard J, Lessig HJ, Black RR. 2500010-overview
Eisenhofer G, Lenders JW, Timmers H, Mannelli M, Grebe SK, Hofbauer LC, et al. Eur J Nucl Med Mol Imaging. Pheochromocytoma: a three-decade clinical experience in a multicenter study. Eur J Endocrinol. 1996 Sep 26. Yalcintepe S, Gurkan H, Korkmaz FN, Demir S, Atli E, Eker D, Guler HS, Zhuri D, Atli EI, Salt SA, Sahin M, Guldiken S. J Kidney Cancer VHL. 2007. Pheochromocytoma treatment guidelines are similar to that of cocaine toxicity. 356(6):601-10. SDHA is a tumor suppressor gene causing paraganglioma. Surg Endosc. [37] : Därr R, Lenders JWM, Hofbauer LC, Naumann B, Bornstein SR, Eisenhofer G. Pheochromocytoma: Update on Disease Management. Bessell-Browne R, O'Malley ME. Hemodynamic instability during resection of pheochromocytoma in MEN versus non-MEN patients. 2002 Mar. 2011 Mar. 2011 Nov. 29(11):2049-60. Surg Endosc. [Medline]. [66] : The ES/AACC/ESE guidelines recommend minimally invasive (eg, laparoscopic) adrenalectomy for most adrenal pheochromocytomas, with open resection reserved for very large or invasive pheochromocytomas. The classic triad consists of headache, hypertension and sweating. Results: Von Hippel–Lindau disease (vHL) is a hereditary tumor predisposition syndrome that places affected individuals at risk for multiple tumors, which are predominantly benign and generally occur in the central nervous system or abdomen. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. Waguespack SG, Rich T, Grubbs E, Ying AK, Perrier ND, Ayala-Ramirez M, et al. [Full Text]. We report a case of complete … diagnosis; guidelines; intra-adrenal paraganglioma; pheochromocytoma; radiology; treatment. 83(2):268-84. George T Griffing, MD Professor Emeritus of Medicine, St Louis University School of Medicine Ann Intern Med. J Endourol. Recent developments in the diagnosis and treatment of pheochromocytoma. 67 (20):2364-74. 150(1):27-32. 99 (6):1915-42. NCCN. Boston, MA -- -- 06/05/2017 -- Global Markets Direct's latest Pharmaceutical and Healthcare disease pipeline guide Pheochromocytoma - Pipeline Review, H1 2017, provides an overview of the Pheochromocytoma (Oncology) pipeline landscape.Pheochromocytoma is a rare, usually non-cancerous (benign) tumor that develops in cells in the center of an adrenal gland. Pheochromocytoma diagnosed during pregnancy: lessons learned from a series of ten patients. Faria JF, Goldman SM, Szejnfeld J, Melo H, Kater C, Kenney P, et al. [66], In patients suspected of having PPGL, biochemical testing via measurement of plasma free metanephrines or urinary fractionated metanephrines is recommended. [Medline]. [Medline]. and there are ∼500 to 1600 new cases in the United States per year . [Medline]. 2020 Jul 2. 2009 Sep. 50(9):1448-54. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. J Headache Pain. [Medline]. [Medline]. The biggest problem for pheochromocytoma is to suspect it in the first place. When the results of these tests are positive and consistent with each other, pheochromocytoma can be diagnosed, and the patient can be submitted to surgery. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. [Full Text]. J Surg Res. Abdominal computed tomography (CT) scan demonstrating left suprarenal mass of soft-tissue attenuation representing a paraganglioma. Gagnon N, Mansour S, Bitton Y, Bourdeau I. TAKOTSUBO-LIKE CARDIOMYOPATHY IN A LARGE COHORT OF PATIENTS WITH PHEOCHROMOCYTOMA AND PARAGANGLIOMA. What is a Pheochromocytoma? 2015 Jun 22;86(ePub):S2239253X15023968. 8600 Rockville Pike [Medline]. Unilateral adrenalectomy in a pregnant patient with bilateral PHEO may be an option to avoid the risk of adrenal insufficiency after bilateral adrenalectomy. [Medline]. Diabetes as a marker of pheochromocytoma in hypertensive patients. Vitamin D for All Over 50s to Prevent Cancer Deaths? In discussing the patient presented here, we provide an update and guidance to help clinicians diagnose pheochromocytoma efficiently and cost-effectively using an algorithmic approach. Hum Mol Genet. Osteoporos Int. 335(13):943-51. 2009 Aug. 94(8):2841-9. 2020 Nov 10;11:587779. doi: 10.3389/fendo.2020.587779. Objective: Radiographics. 2012;3(1):11-26. [Full Text]. Eisenhofer G, Pacak K, Huynh TT, Qin N, Bratslavsky G, Linehan WM, et al. 2004 Oct. 24 Suppl 1:S87-99. International. van den Akker EL, de Krijger RR, de Herder WW, Drop SL. Buitenwerf E, Korteweg T, Visser A, et al. Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. Endocr Pract. Investig Clin Urol. Bibliographies were searched to find additional articles. 329(21):1531-8. [Medline]. 2007 Dec. 245(3):788-97. Multimodality imaging of bilateral pheochromocytoma. Case Report: Pheochromocytoma in a 59-Year-Old Woman Presenting With Hypotension. Clipboard, Search History, and several other advanced features are temporarily unavailable. [Full Text]. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma. In patients with hypertension the prevalence of a PPGL is about 0.2% to 0.6% while in patients with an incidentally discovered adrenal tumor it is about 3% to 7% [ 1, 2, 3 ]. Pheochromocytoma: an imaging chameleon. Niemann S, Becker-Follmann J, Nurnberg G, Ruschendorf F, Sieweke N, et al. Methods: [Medline]. 2010 Aug 1. Acute myocardial infarction attributable to adrenergic crises in a patient with pheochromocytoma and neurofibromatosis 1. J Clin Endocrinol Metab. AJR Am J Roentgenol. is a 53-year-old woman with a history of episodic flushing, hypertension, and an incidentally discovered adrenal mass who presented with shortness of breath, weakness, and lightheadedness in the setting of hypertension to 300/200 mm Hg. Park BK, Kim CK, Kwon GY, Kim JH. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. [Medline]. [Medline]. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors, Version 2.2016. Ferreira VM, Marcelino M, Piechnik SK, et al. Corticothalamic connections of the superior temporal sulcus in rhesus monkeys. J Am Coll Cardiol. [Medline]. Clin Cancer Res. J Clin Endocrinol Metab. [Medline]. [Guideline] National Comprehensive Cancer Network. Medscape Medical News. Elenkova A, Matrozova J, Zacharieva S, Kirilov G, Kalinov K. Adiponectin - A possible factor in the pathogenesis of carbohydrate metabolism disturbances in patients with pheochromocytoma. Dietary influences on plasma and urinary metanephrines: implications for diagnosis of catecholamine-producing tumors. [Medline]. 2013 Feb. 98(2):581-91. [Medline]. [Medline]. Pub Med and Google Scholar databases were searched systematically for studies concerning pheochromocytomas (intra-adrenal paragangliomas) from 1980 until 2016. [Medline]. Elevated metanephrines establish the diagnosis. whereas paragangliomas (PGLs) are neural crest-derived neuroendocrine tumors (NETs) that can originate at any level of extra-adrenal paraganglia (from the skull base to the pelvic floor) Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: a systematic review and meta-analysis. Clinical Case, You are being redirected to
[66], In the immediate postoperative period, the ES/AACC/ESE guidelines recommend monitoring of blood pressure, heart rate, and glucose levels. [Medline]. The RET proto-oncogene in multiple endocrine neoplasia type 2 and Hirschsprung's disease. 1993 Nov 18. Ann T Sweeney, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine SocietyDisclosure: Nothing to disclose. Endocrine tumors such as a pheochromocytoma typically show some degree of nuclear pleomorphism ("endocrine atypia") which does not indicate malignancy.
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