Skip to main content
SpringerLink
Log in
Book cover

Disorders of Blood Pressure Regulation pp 541–560Cite as

Paroxysmal Hypertension: Pheochromocytoma

  • Chapter
  • First Online:

  • 1887 Accesses

Part of the book series: Updates in Hypertension and Cardiovascular Protection ((UHCP))

Abstract

Pheochromocytomas and paragangliomas, arising from respective adrenal and extra-adrenal chromaffin tissue, are infrequent causes of hypertension, but important to consider due to their often fatal nature if undiagnosed. Prevalences vary from less than 2% in patients tested due to hypertension and catecholamine-related symptoms to 7% in patients with incidentalomas and up to 40% in patients with specific hereditary syndromes. Germline and somatic mutations of tumor susceptibility genes are increasingly being recognized as important causes of the tumors that influence disease presentation through differences in activated tumorigenic pathways, including those that control catecholamine biosynthetic and secretory machinery. Biochemical diagnosis is now simplified by measurements of plasma-free metanephrines, the O-methylated metabolites of catecholamines. However, inappropriate application of the test hinders its optimal utility, rendering measurements of urinary fractionated metanephrines more suitable for most nonspecialist centers. When appropriately used, the plasma test not only allows accurate diagnosis but also assessments of underlying mutations, presence of malignancy, as well as tumor size and location. Tumor localization is then usually a simple matter, facilitated also by new functional imaging modalities, choice of which can benefit from consideration of underlying mutations. Management and treatment continues to rely on preoperative blockade of the effects of catecholamines, with surgical intervention usually but not always offering cure. Due to risks of postoperative recurrence, including metastatic involvement, long-term follow-up is important, with increasing indications that the nature of this should be personalized according to underling mutations, as well as size, location, and biochemical features of resected primary tumors.

This is a preview of subscription content, log in via an institution.

References

  1. Tischler AS (2008) Pheochromocytoma and extra-adrenal paraganglioma: updates. Arch Pathol Lab Med 132(8):1272–1284. doi:2007-0731-RAR [pii]

    Google Scholar 

  2. Waguespack SG, Rich T, Grubbs E et al (2010) A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 95(5):2023–2037. doi:10.1210/jc.2009-2830

    Article  CAS  PubMed  Google Scholar 

  3. Manger WM (2009) The protean manifestations of pheochromocytoma. Hom Metab Res 41(9):658–663. doi:10.1055/s-0028-1128139

    Article  CAS  Google Scholar 

  4. Mannelli M, Lenders JW, Pacak K et al (2012) Subclinical phaeochromocytoma. Best Pract Res Clin Endocrinol Metab 26(4):507–515. doi:10.1016/j.beem.2011.10.008

    Article  PubMed  PubMed Central  Google Scholar 

  5. Sutton MG, Sheps SG, Lie JT (1981) Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. Mayo Clin Proc 56(6):354–360

    CAS  PubMed  Google Scholar 

  6. McNeil AR, Blok BH, Koelmeyer TD et al (2000) Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland. Aust NZ J Med 30(6):648–652

    Article  CAS  Google Scholar 

  7. Lo CY, Lam KY, Wat MS, Lam KS (2000) Adrenal pheochromocytoma remains a frequently overlooked diagnosis. Am J Surg 179(3):212–215

    Article  CAS  PubMed  Google Scholar 

  8. Fernandez-Calvet L, Garcia-Mayor RV (1994) Incidence of pheochromocytoma in South Galicia, Spain. J Intern Med 236(6):675–677

    Article  CAS  PubMed  Google Scholar 

  9. Ariton M, Juan CS, AvRuskin TW (2000) Pheochromocytoma: clinical observations from a Brooklyn tertiary hospital. Endocr Pract 6(3):249–252

    Article  CAS  PubMed  Google Scholar 

  10. Omura M, Saito J, Yamaguchi K et al (2004) Prospective study on the prevalence of secondary hypertension among hypertensive patients visiting a general outpatient clinic in Japan. Hypertens Res 27(3):193–202

    Article  PubMed  Google Scholar 

  11. Hernandez FC, Sanchez M, Alvarez A et al (2000) A five-year report on experience in the detection of pheochromocytoma. Clin Biochem 33(8):649–655

    Article  CAS  PubMed  Google Scholar 

  12. Vaclavik J, Stejskal D, Lacnak B et al (2007) Free plasma metanephrines as a screening test for pheochromocytoma in low-risk patients. J Hypertens 25(7):1427–1431

    Article  CAS  PubMed  Google Scholar 

  13. Brain KL, Kay J, Shine B (2006) Measurement of urinary metanephrines to screen for pheochromocytoma in an unselected hospital referral population. Clin Chem 52(11):2060–2064. doi:10.1373/clinchem.2006.070805

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Mantero F, Terzolo M, Arnaldi G et al (2000) A survey on adrenal incidentaloma in Italy. Study group on adrenal Tumors of the Italian Society of endocrinology. J Clin Endocrinol Metab 85(2):637–644

    CAS  PubMed  Google Scholar 

  15. Terzolo M, Bovio S, Pia A et al (2009) Management of adrenal incidentaloma. Best Pract Res Clin Endocrinol Metab 23(2):233–243. doi:10.1016/j.beem.2009.04.001

    Article  PubMed  Google Scholar 

  16. Mansmann G, Lau J, Balk E et al (2004) The clinically inapparent adrenal mass: update in diagnosis and management. Endocrine Rev 25(2):309–340

    Article  Google Scholar 

  17. Howe JR, Norton JA, Wells SA Jr (1993) Prevalence of pheochromocytoma and hyperparathyroidism in multiple endocrine neoplasia type 2A: results of long-term follow-up. Surgery 114(6):1070–1077

    CAS  PubMed  Google Scholar 

  18. Eisenhofer G, Goldstein DS, Sullivan P et al (2005) Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab 90:2068–2075

    Article  CAS  PubMed  Google Scholar 

  19. Timmers HJ, Pacak K, Huynh TT et al (2008) Biochemically silent abdominal Paragangliomas in patients with mutations in the Sdhb Gene. J Clin Endocrinol Metab 93:4826–4832

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Zelinka T, Strauch B, Petrak O et al (2005) Increased blood pressure variability in pheochromocytoma compared to essential hypertension patients. J Hypertens 23(11):2033–2039. doi:00004872-200511000-00018 [pii]

    Article  CAS  PubMed  Google Scholar 

  21. Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A (2011) Cardiovascular manifestations of phaeochromocytoma. J Hypertens 29(11):2049–2060. doi:10.1097/HJH.0b013e32834a4ce9

    Article  CAS  PubMed  Google Scholar 

  22. Bergland BE (1989) Pheochromocytoma presenting as shock. Am J Emerg Med 7(1):44–48

    Article  CAS  PubMed  Google Scholar 

  23. Brouwers FM, Eisenhofer G, Lenders JW, Pacak K (2006) Emergencies caused by pheochromocytoma, neuroblastoma, or ganglioneuroma. Endocrinol Metab Clin N Am 35(4):699–724

    Article  CAS  Google Scholar 

  24. Giavarini A, Chedid A, Bobrie G et al (2013) Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma. Heart ;99(19):1438–1444. doi: 10.1136/heartjnl-2013-304073

  25. Riester A, Weismann D, Quinkler M et al (2015) Life-threatening events in patients with pheochromocytoma. Eur J Endocrinol 173(6):757–764. doi:10.1530/EJE-15-0483

    Article  CAS  PubMed  Google Scholar 

  26. Lance JW, Hinterberger H (1976) Symptoms of pheochromocytoma, with particular reference to headache, correlated with catecholamine production. Arch Neurol 33(4):281–288

    Article  CAS  PubMed  Google Scholar 

  27. Ito Y, Fujimoto Y, Obara T (1992) The role of epinephrine, norepinephrine, and dopamine in blood pressure disturbances in patients with pheochromocytoma. World J Surg 16(4):759–763. discussion 763-754

    Article  CAS  PubMed  Google Scholar 

  28. Eisenhofer G, Walther MM, Huynh TT et al (2001) Pheochromocytomas in von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes. J Clin Endocrinol Metab 86(5):1999–2008

    Article  CAS  PubMed  Google Scholar 

  29. Proye C, Fossati P, Fontaine P et al (1986) Dopamine-secreting pheochromocytoma: an unrecognized entity? Classification of pheochromocytomas according to their type of secretion. Surgery 100(6):1154–1162

    CAS  PubMed  Google Scholar 

  30. Awada SH, Grisham A, Woods SE (2003) Large dopamine-secreting pheochromocytoma: case report. South Med J 96(9):914–917

    Article  PubMed  Google Scholar 

  31. Lenders JW, Eisenhofer G, Mannelli M, Pacak K (2005) Phaeochromocytoma. Lancet 366(9486):665–675

    Article  PubMed  Google Scholar 

  32. Amar L, Servais A, Gimenez-Roqueplo AP et al (2005) Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma. J Clin Endocrinol Metab 90(4):2110–2116

    Article  CAS  PubMed  Google Scholar 

  33. Kopetschke R, Slisko M, Kilisli A et al (2009) Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol 161(2):355–361. doi:10.1530/EJE-09-0384

    Article  CAS  PubMed  Google Scholar 

  34. Terzolo M, Pia A, Ali A et al (2002) Adrenal incidentaloma: a new cause of the metabolic syndrome? J Clin Endocrinol Metab 87(3):998–1003

    Article  CAS  PubMed  Google Scholar 

  35. Kirshtein B, Pagliarello G, Yelle JD, Poulin EC (2007) Incidence of pheochromocytoma in trauma patients during the management of unrelated illness: a retrospective review. Int J Surg 5(5):332–335. doi:10.1016/j.ijsu.2007.04.015

    Article  CAS  PubMed  Google Scholar 

  36. Haissaguerre M, Courel M, Caron P et al (2013) Normotensive incidentally discovered pheochromocytomas display specific biochemical, cellular, and molecular characteristics. J Clin Endocrinol Metab 98(11):4346–4354. doi:10.1210/jc.2013-1844

    Article  CAS  PubMed  Google Scholar 

  37. Dahia PL (2014) Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity. Nat Rev Cancer 14(2):108–119. doi:10.1038/nrc3648

    Article  CAS  PubMed  Google Scholar 

  38. Flynn A, Benn D, Clifton-Bligh R et al (2015) The genomic landscape of phaeochromocytoma. J Pathol 236(1):78–89. doi:10.1002/path.4503

    Article  CAS  PubMed  Google Scholar 

  39. Walther MM, Herring J, Enquist E et al (1999) von Recklinghausen’s disease and pheochromocytomas. J Urol 162(5):1582–1586

    Article  CAS  PubMed  Google Scholar 

  40. Walther MM, Keiser HR, Choyke PL et al (1999) Management of hereditary pheochromocytoma in von Hippel-Lindau kindreds with partial adrenalectomy. J Urol 161(2):395–398

    Article  CAS  PubMed  Google Scholar 

  41. Peczkowska M, Erlic Z, Hoffmann MM et al (2008) Impact of screening kindreds for SDHD p.Cys11X as a common mutation associated with paraganglioma syndrome type 1. J Clin Endocrinol Metab 93(12):4818–4825. doi:10.1210/jc.2008-1290

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Baysal BE (2004) Genomic imprinting and environment in hereditary paraganglioma. Am J Med Genet C Semin Med Genet 129C(1):85–90

    Article  PubMed  Google Scholar 

  43. Comino-Méndez I, Gracia-Aznárez FJ, Schiavi F et al (2011) Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma. Nat Genet 43:663–667

    Article  PubMed  Google Scholar 

  44. Hoekstra AS, Devilee P, Bayley JP (2015) Models of parent-of-origin tumorigenesis in hereditary paraganglioma. Semin Cell Dev Biol 43:117–124. doi:10.1016/j.semcdb.2015.05.011

    Article  PubMed  Google Scholar 

  45. van Duinen N, Steenvoorden D, Bonsing BA et al (2010) Pheochromocytomas detected by biochemical screening in predisposed subjects are associated with lower prevalence of clinical and biochemical manifestations and smaller tumors than pheochromocytomas detected by signs and symptoms. Eur J Endocrinol 163(1):121–127. doi:10.1530/EJE-10-0114

    Article  PubMed  Google Scholar 

  46. Amar L, Bertherat J, Baudin E et al (2005) Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol 23(34):8812–8818

    Article  CAS  PubMed  Google Scholar 

  47. Lenders JWM, Duh QY, Eisenhofer G et al (2014) Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 99:1915–1942

    Article  CAS  PubMed  Google Scholar 

  48. Favier J, Amar L, Gimenez-Roqueplo AP (2015) Paraganglioma and phaeochromocytoma: from genetics to personalized medicine. Nature Rev Endocrinol 11(2):101–111. doi:10.1038/nrendo.2014.188

    Article  CAS  Google Scholar 

  49. Jochmanova I, Yang C, Zhuang Z, Pacak K (2013) Hypoxia-inducible factor signaling in pheochromocytoma: turning the rudder in the right direction. J Natl Canc Inst 105(17):1270–1283. doi:10.1093/jnci/djt201

    Article  CAS  Google Scholar 

  50. Qin N, de Cubas AA, Garcia-Martin R et al (2014) Opposing effects of HIF1alpha and HIF2alpha on chromaffin cell phenotypic features and tumor cell proliferation: insights from MYC-associated factor X. Int J Cancer 135(9):2054–2064. doi:10.1002/ijc.28868

    Article  CAS  PubMed  Google Scholar 

  51. Eisenhofer G, Huynh TT, Pacak K et al (2004) Distinct gene expression profiles in norepinephrine- and epinephrine-producing hereditary and sporadic pheochromocytomas: activation of hypoxia-driven angiogenic pathways in von Hippel-Lindau syndrome. Endocr Relat Cancer 11:897–911

    Article  CAS  PubMed  Google Scholar 

  52. Eisenhofer G, Timmers H, Lenders JW et al (2011) Age at diagnosis of pheochromocytoma differs according to catecholamine phenotype and tumor location. J Clin Endocrinol Metab 96:375–384

    Article  CAS  PubMed  Google Scholar 

  53. Richter S, Qin N, Pacak K, Eisenhofer G (2013) Role of hypoxia and HIF2a in development of the sympathoadrenal cell lineage and chromaffin cell tumors with distinct catecholamine phenotypic features. Adv Pharmacol 68:285–317

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. Zhuang Z, Yang C, Lorenzo F et al (2012) Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia. NEJM 367(10):922–930. doi:10.1056/NEJMoa1205119

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Buffet A, Smati S, Mansuy L et al (2014) Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome. J Clin Endocrinol Metab 99(2):E369–E373. doi:10.1210/jc.2013-2600

    Article  CAS  PubMed  Google Scholar 

  56. Letouze E, Martinelli C, Loriot C et al (2013) SDH mutations establish a hypermethylator phenotype in paraganglioma. Cancer Cell 23(6):739–752. doi:10.1016/j.ccr.2013.04.018

    Article  CAS  PubMed  Google Scholar 

  57. Eisenhofer G, Huynh TT, Elkahloun A et al (2008) Differential expression of the regulated catecholamine secretory pathway in different hereditary forms of pheochromocytoma. Am J Physiol Endocrinol Metab 295(5):E1223–E1233

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. Eisenhofer G, Pacak K, Huynh TT et al (2011) Catecholamine metabolomic and secretory phenotypes in phaeochromocytoma. Endocr Relat Cancer 18(1):97–111. doi:ERC-10-0211 [pii]. doi:10.1677/ERC-10-0211

    Article  CAS  PubMed  Google Scholar 

  59. Eisenhofer G, Lenders JW, Siegert G et al (2012) Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer 48:1739–1749

    Article  CAS  PubMed  Google Scholar 

  60. Feldman JM (1985) Increased dopamine production in patients with carcinoid tumors. Metab Clin Exp 34(3):255–260

    Article  CAS  PubMed  Google Scholar 

  61. Eisenhofer G, Kopin IJ, Goldstein DS (2004) Leaky catecholamine stores: undue waste or a stress response coping mechanism? Ann N Y Acad Sci 1018:224–230

    Article  CAS  PubMed  Google Scholar 

  62. Eisenhofer G, Kopin IJ, Goldstein DS (2004) Catecholamine metabolism: a contemporary view with implications for physiology and medicine. Pharmacol Rev 56(3):331–349

    Article  CAS  PubMed  Google Scholar 

  63. Eisenhofer G, Keiser H, Friberg P et al (1998) Plasma metanephrines are markers of pheochromocytoma produced by catechol-O-methyltransferase within tumors. J Clin Endocrinol Metab 83(6):2175–2185

    Article  CAS  PubMed  Google Scholar 

  64. Eisenhofer G, Rundquist B, Aneman A et al (1995) Regional release and removal of catecholamines and extraneuronal metabolism to metanephrines. J Clin Endocrinol Metab 80(10):3009–3017

    CAS  PubMed  Google Scholar 

  65. Eisenhofer G, Aneman A, Hooper D et al (1996) Mesenteric organ production, hepatic metabolism, and renal elimination of norepinephrine and its metabolites in humans. J Neurochem 66(4):1565–1573

    Article  PubMed  Google Scholar 

  66. Lenders JW, Pacak K, Walther MM et al (2002) Biochemical diagnosis of pheochromocytoma: which test is best? JAMA 287(11):1427–1434

    Article  CAS  PubMed  Google Scholar 

  67. Raber W, Raffesberg W, Bischof M et al (2000) Diagnostic efficacy of unconjugated plasma metanephrines for the detection of pheochromocytoma. Arch Intern Med 160(19):2957–2963

    Google Scholar 

  68. Unger N, Pitt C, Schmidt IL et al (2006) Diagnostic value of various biochemical parameters for the diagnosis of pheochromocytoma in patients with adrenal mass. Eur J Endocrinol 154(3):409–417

    Article  CAS  PubMed  Google Scholar 

  69. Hickman PE, Leong M, Chang J et al (2009) Plasma free metanephrines are superior to urine and plasma catecholamines and urine catecholamine metabolites for the investigation of phaeochromocytoma. Pathology 41(2):173–177. doi:907871493 [pii]. doi:10.1080/00313020802579284

    Article  CAS  PubMed  Google Scholar 

  70. Grouzmann E, Drouard-Troalen L, Baudin E et al (2010) Diagnostic accuracy of free and total metanephrines in plasma and fractionated metanephrines in urine of patients with pheochromocytoma. Eur J Endocrinol 162(5):951–960. doi:EJE-09-0996 [pii]. doi:10.1530/EJE-09-0996

    Article  CAS  PubMed  Google Scholar 

  71. Pussard E, Chaouch A, Said T (2014) Radioimmunoassay of free plasma metanephrines for the diagnosis of catecholamine-producing tumors. Clin Chem Lab Med 52(3):437–444. doi:10.1515/cclm-2013-0406

    Article  CAS  PubMed  Google Scholar 

  72. Procopiou M, Finney H, Akker SA et al (2009) Evaluation of an enzyme immunoassay for plasma-free metanephrines in the diagnosis of catecholamine-secreting tumors. Eur J Endocrinol 161(1):131–140. doi:EJE-09-0172 [pii]. doi:10.1530/EJE-09-0172

    Article  CAS  PubMed  Google Scholar 

  73. Christensen TT, Frystyk J, Poulsen PL (2011) Comparison of plasma metanephrines measured by a commercial immunoassay and urinary catecholamines in the diagnosis of pheochromocytoma. Scan J Clin Lab Invest 71(8):695–700. doi:10.3109/00365513.2011.622410

    Article  CAS  Google Scholar 

  74. Darr R, Pamporaki C, Peitzsch M et al (2014) Biochemical diagnosis of phaeochromocytoma using plasma-free normetanephrine, metanephrine and methoxytyramine: importance of supine sampling under fasting conditions. Clin Endocrinol 80(4):478–486. doi:10.1111/cen.12327

    Article  Google Scholar 

  75. Eisenhofer G, Peitzsch M (2014) Laboratory evaluation of pheochromocytoma and paraganglioma. Clin Chem 60(12):1486–1499. doi:10.1373/clinchem.2014.224832

    Article  CAS  PubMed  Google Scholar 

  76. Eisenhofer G, Lenders JW, Timmers H et al (2011) Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma. Clin Chem 57(3):411–420. doi:clinchem.2010.153320 [pii]. doi:10.1373/clinchem.2010.153320

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  77. Eisenhofer G, Lenders JW, Goldstein DS et al (2005) Pheochromocytoma catecholamine phenotypes and prediction of tumor size and location by use of plasma free metanephrines. Clin Chem 51(4):735–744

    Article  CAS  PubMed  Google Scholar 

  78. Amar L, Eisenhofer G (2015) Diagnosing phaeochromocytoma/paraganglioma in a patient presenting with critical illness: biochemistry versus imaging. Clin Endocrinol 83(3):298–302. doi:10.1111/cen.12745

    Article  Google Scholar 

  79. IliasI, SahdevA, ReznekRHet al (2007) The optimal imaging of adrenal tumours: a comparison of different methods. Endocr Relat Cancer 14 (3):587-599. doi:14/3/587[pii]10.1677/ERC-07-0045

  80. Sahdev A, Sohaib A, Monson JP et al (2005) CT and MR imaging of unusual locations of extra-adrenal paragangliomas (pheochromocytomas). Eur Radiol 15(1):85–92

    Article  PubMed  Google Scholar 

  81. Leung K, Stamm M, Raja A, Low G (2013) Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging. AJR Am J Roentgenol 200(2):370–378. doi:10.2214/AJR.12.9126

    Article  PubMed  Google Scholar 

  82. Pacak K, Eisenhofer G, Goldstein DS (2004) Functional imaging of endocrine tumors: role of positron emission tomography. Endocr Rev 25(4):568–580

    Article  PubMed  Google Scholar 

  83. Taieb D, Timmers HJ, Hindie E et al (2012) EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging 39(12):1977–1995. doi:10.1007/s00259-012-2215-8

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  84. Wiseman GA, Pacak K, O’Dorisio MS et al (2009) 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. J Nucl Med 50(9):1448–1454. doi:jnumed.108.058701 [pii]. doi:10.2967/jnumed.108.058701

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  85. Fiebrich HB, Brouwers AH, Kerstens MN et al (2009) 6-[F-18]Fluoro-L-dihydroxyphenylalanine positron emission tomography is superior to conventional imaging with (123)I-metaiodobenzylguanidine scintigraphy, computer tomography, and magnetic resonance imaging in localizing tumors causing catecholamine excess. J Clin Endocrinol Metab 94(10):3922–3930. doi:10.1210/jc.2009-1054

    Article  CAS  PubMed  Google Scholar 

  86. Timmers HJ, Chen CC, Carrasquillo JA et al (2012) Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography. J Natl Canc Inst 104(9):700–708. doi:10.1093/jnci/djs188

    Article  CAS  Google Scholar 

  87. Maurice JB, Troke R, Win Z et al (2012) A comparison of the performance of (6)(8)Ga-DOTATATE PET/CT and (1)(2)(3)I-MIBG SPECT in the diagnosis and follow-up of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging 39(8):1266–1270. doi:10.1007/s00259-012-2119-7

    Article  CAS  PubMed  Google Scholar 

  88. Janssen I, Blanchet EM, Adams K et al (2015) Superiority of [68Ga]-DOTATATE PET/CT to other functional imaging modalities in the localization of SDHB-associated metastatic Pheochromocytoma and Paraganglioma. Clin Cancer Res 21(17):3888–3895. doi:10.1158/1078-0432.CCR-14-2751

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  89. Janssen I, Chen CC, Taieb D et al (2016) 68Ga-DOTATATE PET/CT in the localization of head and neck Paragangliomas compared with other functional imaging modalities and CT/MRI. J Nucl Med 57(2):186–191. doi:10.2967/jnumed.115.161018

    Article  CAS  PubMed  Google Scholar 

  90. Amar L, Fassnacht M, Gimenez-Roqueplo AP et al (2012) Long-term postoperative follow-up in patients with apparently benign pheochromocytoma and paraganglioma. Hom Metab Res 44(5):385–389

    Google Scholar 

  91. Pacak K, Eisenhofer G, Ahlman H et al (2007) Pheochromocytoma: recommendations for clinical practice from the first international symposium. Nat Clin Pract Endocrinol Metab 3(2):92–102

    Article  PubMed  Google Scholar 

  92. Weingarten TN, Cata JP, O’Hara JF et al (2010) Comparison of two preoperative medical management strategies for laparoscopic resection of pheochromocytoma. Urology 76(2):508e506–511. doi: 10.1016/j.urology.2010.03.032

  93. Steinsapir J, Carr AA, Prisant LM, Bransome ED Jr (1997) Metyrosine and pheochromocytoma. Arch Int Med 157(8):901–906

    Article  CAS  Google Scholar 

  94. Walz MK, Alesina PF, Wenger FA et al (2006) Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients. World J Surg 30(5):899–908. doi:10.1007/s00268-005-0373-6

    Article  PubMed  Google Scholar 

  95. Goers TA, Abdo M, Moley JF et al (2013) Outcomes of resection of extra-adrenal pheochromocytomas/paragangliomas in the laparoscopic era: a comparison with adrenal pheochromocytoma. Surg Endosc 27(2):428–433. doi:10.1007/s00464-012-2451-9

    Article  PubMed  Google Scholar 

  96. Shen WT, Grogan R, Vriens M et al (2010) One hundred two patients with pheochromocytoma treated at a single institution since the introduction of laparoscopic adrenalectomy. Arch Surg 145(9):893–897. doi:10.1001/archsurg.2010.159

    Article  PubMed  Google Scholar 

  97. Agarwal G, Sadacharan D, Aggarwal V et al (2012) Surgical management of organ-contained unilateral pheochromocytoma: comparative outcomes of laparoscopic and conventional open surgical procedures in a large single-institution series. Langenbeck’s Arch Surg 397(7):1109–1116. doi:10.1007/s00423-011-0879-3

    Article  Google Scholar 

  98. Asari R, Scheuba C, Kaczirek K, Niederle B (2006) Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A. Arch Surg 141(12):1199–1205.; discussion 1205. doi:10.1001/archsurg.141.12.1199

    Article  CAS  PubMed  Google Scholar 

  99. Benhammou JN, Boris RS, Pacak K et al (2010) Functional and oncologic outcomes of partial adrenalectomy for pheochromocytoma in patients with von Hippel-Lindau syndrome after at least 5 years of followup. J Urol 184(5):1855–1859. doi:10.1016/j.juro.2010.06.102

    Article  PubMed  PubMed Central  Google Scholar 

  100. Grubbs EG, Rich TA, Ng C et al (2013) Long-term outcomes of surgical treatment for hereditary pheochromocytoma. J Am Coll Surg 216(2):280–289. doi:10.1016/j.jamcollsurg.2012.10.012

    Article  PubMed  Google Scholar 

  101. Plouin PF, Amar L, Dekkers OM et al (2016) European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol 174(5):G1–G10. doi:10.1530/EJE-16-0033

    Article  CAS  PubMed  Google Scholar 

  102. Amar L, Lussey-Lepoutre C, Lenders J et al (2016) Management of endocrine disease: recurrence or new tumors after complete resection of phaeochromocytomas and paragangliomas. A systematic review and meta-analysis. Eur J Endocrinol. 175(4):R135–R145. doi: 10.1530/EJE-16-0189

Download references

Author information

Authors and Affiliations

  1. Institute of Clinical Chemistry and Laboratory Medicine and Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

    Graeme Eisenhofer

  2. Department of Internal Medicine, Division of Vascular Medicine, Radboud University Medical Center, Nijmegen, The Netherlands

    Jacques W. M. Lenders

  3. Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

    Jacques W. M. Lenders

Authors
  1. Graeme Eisenhofer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jacques W. M. Lenders
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Graeme Eisenhofer .

Editor information

Editors and Affiliations

  1. Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon

    Adel E. Berbari

  2. Emeritus Professor of Medicine, University of Milano-Bicocca, Monza, Italy

    Giuseppe Mancia

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Cite this chapter

Eisenhofer, G., Lenders, J.W.M. (2018). Paroxysmal Hypertension: Pheochromocytoma. In: Berbari, A., Mancia, G. (eds) Disorders of Blood Pressure Regulation. Updates in Hypertension and Cardiovascular Protection. Springer, Cham. https://doi.org/10.1007/978-3-319-59918-2_31

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-59918-2_31

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59917-5

  • Online ISBN: 978-3-319-59918-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation