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Pheochromocytoma and Paraganglioma

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Neuroendocrine Tumours

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Abstract

Paragangliomas (PGLs) are rare vascular, neuroendocrine tumors of paraganglia. They can occur sporadically or hereditary, due to germ-line mutations in one of 10 susceptibility genes. PGLs derive from either sympathetic tissue in adrenal (pheochromocytomas or PCCs) and extra-adrenal (sPGLs) locations or parasympathetic tissue of the head and neck (HNPGLs). Since HNPGLs are usually benign and most tumors grow slowly, a wait-and-scan policy is often advised. However, their location in the close proximity to nerves and vasculature can result in considerable morbidity due to compression or infiltration of the adjacent structures. The main treatment modalities for HNPGL are surgery and radiotherapy.

The majority of PCCs and sPGLs produce catecholamines, with hypertension as the most common feature. To detect these tumors, plasma-free metanephrines and/or 24 h excretion of urinary fractionated metanephrines can be measured. Once a biochemical diagnosis of sPGL/PCC has been established, the source of catecholamine excess is subsequently localized by anatomical (computed tomography and/or magnetic resonance imaging) and functional imaging studies (123I-MIBG-scintigraphy, 111In-pentetreotide scintigraphy, or positron emission tomography with radiolabeled dopamine or dihydroxyphenylalanine). The treatment of choice for PCCs and sPGLs is surgical resection after adequate pretreatment with α-blockade, if necessary followed by β-blockade, and volume expansion.

Nearly 10 % of PCCs and 10–20 % of sPGLs are malignant. Genetic testing can be of great importance for patients and their relatives because it gives the opportunity for early diagnosis through periodical imaging studies in the affected individual and family members. Early detection of a familial PGL allows early detection of potentially malignant PGLs and early surgical treatment, reducing the complication rate of this operation.

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References

  1. De Lellis RA, Lloyd RV, Heitz PU, Eng C (2004) Pathology and genetics of tumours of endocrine organs, World Health Organization Classification of Tumours. IARC Press, Lyon

    Google Scholar 

  2. Pacak K, Keiser H, Eisenhofer G (2005) Pheochromocytoma. In: DeGroot LS, Jameson JL (eds) Textbook of endocrinology, 5th edn. Elsevier Saunders Inc, Philadelphia, pp 2501–2534

    Google Scholar 

  3. Kliewer KE, Wen DR, Cancilla PA, Cochran AJ (1989) Paragangliomas: assessment of prognosis by histologic, immunohistochemical, and ultrastructural techniques. Hum Pathol 20:29–39

    CAS  PubMed  Google Scholar 

  4. Sennaroglu L, Sungur A (2002) Histopathology of paragangliomas. Otol Neurotol 23:104–105

    PubMed  Google Scholar 

  5. Warren WH, Lee I, Gould VE, Memoli VA, Jao W (1985) Paragangliomas of the head and neck: ultrastructural and immunohistochemical analysis. Ultrastruct Pathol 8:333–343

    CAS  PubMed  Google Scholar 

  6. Lack EE, Cubilla AL, Woodruff JM (1979) Paragangliomas of the head and neck region. A pathologic study of tumors from 71 patients. Hum Pathol 10:191–218

    CAS  PubMed  Google Scholar 

  7. Granger JK, Houn HY (1990) Head and neck paragangliomas: a clinicopathologic study with DNA flow cytometric analysis. South Med J 83:1407–1412

    CAS  PubMed  Google Scholar 

  8. Scholz T, Schulz C, Klose S, Lehnert H (2007) Diagnostic management of benign and malignant pheochromocytoma. Exp Clin Endocrinol Diabetes 115:155–159

    CAS  PubMed  Google Scholar 

  9. Amar L, Baudin E, Burnichon N et al (2007) Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas. J Clin Endocrinol Metab 92:3822–3828

    CAS  PubMed  Google Scholar 

  10. Strong VE, Kennedy T, Al-Ahmadie H et al (2008) Prognostic indicators of malignancy in adrenal pheochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery 143:759–768

    PubMed  Google Scholar 

  11. Liu TH, Chen YJ, Wu SF et al (2004) Distinction between benign and malignant pheochromocytomas. Zhonghua Bing Li Xue Za Zhi 33:198–202

    PubMed  Google Scholar 

  12. Chen H, Sippel RS, O’Dorisio MS et al (2010) The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 39:775–783

    PubMed Central  PubMed  Google Scholar 

  13. McNeil AR, Blok BH, Koelmeyer TD, Burke MP, Hilton JM (2000) Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland. Aust N Z J Med 30:648–652

    CAS  PubMed  Google Scholar 

  14. Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT (1983) Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc 58:802–804

    CAS  PubMed  Google Scholar 

  15. Cascon A, Pita G, Burnichon N et al (2009) Genetics of pheochromocytoma and paraganglioma in Spanish patients. J Clin Endocrinol Metab 94:1701–1705

    CAS  PubMed  Google Scholar 

  16. Mannelli M, Castellano M, Schiavi F et al (2009) Clinically guided genetic screening in a large cohort of Italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas. J Clin Endocrinol Metab 94:1541–1547

    CAS  PubMed  Google Scholar 

  17. Jansen JC, van den Berg R, Kuiper A et al (2000) Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 88:2811–2816

    CAS  PubMed  Google Scholar 

  18. van Duinen N, Steenvoorden D, Kema IP et al (2010) Increased urinary excretion of 3-methoxytyramine in patients with head and neck paragangliomas. J Clin Endocrinol Metab 95:209–214

    PubMed  Google Scholar 

  19. Gardner P, Dalsing M, Weisberger E, Sawchuk A, Miyamoto R (1996) Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas. Am J Surg 172:196–199

    CAS  PubMed  Google Scholar 

  20. Netterville JL, Jackson CG, Miller FR, Wanamaker JR, Glasscock ME (1998) Vagal paraganglioma: a review of 46 patients treated during a 20-year period. Arch Otolaryngol Head Neck Surg 124:1133–1140

    CAS  PubMed  Google Scholar 

  21. Jackson CG (2001) Glomus tympanicum and glomus jugulare tumors. Otolaryngol Clin North Am 34:941–970, vii

    CAS  PubMed  Google Scholar 

  22. Bravo EL (1994) Evolving concepts in the pathophysiology, diagnosis, and treatment of pheochromocytoma. Endocr Rev 15:356–368

    CAS  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  24. Kebebew E, Duh QY (1998) Benign and malignant pheochromocytoma: diagnosis, treatment, and follow-up. Surg Oncol Clin N Am 7:765–789

    CAS  PubMed  Google Scholar 

  25. Werbel SS, Ober KP (1995) Pheochromocytoma. Update on diagnosis, localization, and management. Med Clin North Am 79:131–153

    CAS  PubMed  Google Scholar 

  26. La Batide-Alanore A, Chatellier G, Plouin PF (2003) Diabetes as a marker of pheochromocytoma in hypertensive patients. J Hypertens 21:1703–1707

    PubMed  Google Scholar 

  27. Pacak K, Eisenhofer G, Ahlman H et al (2007) Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab 3:92–102

    PubMed  Google Scholar 

  28. Neumann HP, Erlic Z, Boedeker CC et al (2009) Clinical predictors for germline mutations in head and neck paraganglioma patients: cost reduction strategy in genetic diagnostic process as fall-out. Cancer Res 69:3650–3656

    CAS  PubMed  Google Scholar 

  29. Latif F, Tory K, Gnarra J et al (1993) Identification of the von Hippel-Lindau disease tumor suppressor gene. Science 260:1317–1320

    CAS  PubMed  Google Scholar 

  30. Eng C (1996) Seminars in medicine of the Beth Israel Hospital, Boston. The RET proto-oncogene in multiple endocrine neoplasia type 2 and Hirschsprung’s disease. N Engl J Med 335:943–951

    CAS  PubMed  Google Scholar 

  31. Neumann HP, Berger DP, Sigmund G et al (1993) Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med 329:1531–1538

    CAS  PubMed  Google Scholar 

  32. White R, O’Connell P (1991) Identification and characterization of the gene for neurofibromatosis type 1. Curr Opin Genet Dev 1:15–19

    CAS  PubMed  Google Scholar 

  33. Neumann HP, Bausch B, McWhinney SR et al (2002) Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 346:1459–1466

    CAS  PubMed  Google Scholar 

  34. Astuti D, Latif F, Dallol A et al (2001) Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet 69:49–54

    PubMed Central  CAS  PubMed  Google Scholar 

  35. Baysal BE, Ferrell RE, Willett-Brozick JE et al (2000) Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Science 287:848–851

    CAS  PubMed  Google Scholar 

  36. Burnichon N, Briere JJ, Libe R et al (2010) SDHA is a tumor suppressor gene causing paraganglioma. Hum Mol Genet 19:3011–3020

    PubMed Central  CAS  PubMed  Google Scholar 

  37. Niemann S, Muller U (2000) Mutations in SDHC cause autosomal dominant paraganglioma, type 3. Nat Genet 26:268–270

    CAS  PubMed  Google Scholar 

  38. Hao HX, Khalimonchuk O, Schraders M et al (2009) SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma. Science 325:1139–1142

    CAS  PubMed  Google Scholar 

  39. Qin Y, Yao L, King EE et al (2010) Germline mutations in TMEM127 confer susceptibility to pheochromocytoma. Nat Genet 42:229–233

    PubMed Central  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  41. Gimenez-Roqueplo AP, Dahia PL, Robledo M (2012) An update on the genetics of paraganglioma, pheochromocytoma, and associated hereditary syndromes. Horm Metab Res 44:328–333

    CAS  PubMed  Google Scholar 

  42. van Hulsteijn LT, Dekkers OM, Hes FJ, Smit JW, Corssmit EP (2012) Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis. J Med Genet 49:768–776

    PubMed  Google Scholar 

  43. Welander J, Soderkvist P, Gimm O (2011) Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas. Endocr Relat Cancer 18:R253–R276

    CAS  PubMed  Google Scholar 

  44. Carney JA, Stratakis CA (2002) Familial paraganglioma and gastric stromal sarcoma: a new syndrome distinct from the Carney triad. Am J Med Genet 108:132–139

    PubMed  Google Scholar 

  45. Schussheim DH, Skarulis MC, Agarwal SK et al (2001) Multiple endocrine neoplasia type 1: new clinical and basic findings. Trends Endocrinol Metab 12:173–178

    CAS  PubMed  Google Scholar 

  46. McWhinney SR, Pasini B, Stratakis CA (2007) Familial gastrointestinal stromal tumors and germ-line mutations. N Engl J Med 357:1054–1056

    CAS  PubMed  Google Scholar 

  47. Vanharanta S, Buchta M, McWhinney SR et al (2004) Early-onset renal cell carcinoma as a novel extraparaganglial component of SDHB-associated heritable paraganglioma. Am J Hum Genet 74:153–159

    PubMed Central  CAS  PubMed  Google Scholar 

  48. Xekouki P, Pacak K, Almeida M et al (2012) Succinate dehydrogenase (SDH) D subunit (SDHD) inactivation in a growth-hormone-producing pituitary tumor: a new association for SDH? J Clin Endocrinol Metab 97:E357–E366

    PubMed Central  CAS  PubMed  Google Scholar 

  49. Baysal BE, Farr JE, Rubinstein WS et al (1997) Fine mapping of an imprinted gene for familial nonchromaffin paragangliomas, on chromosome 11q23. Am J Hum Genet 60:121–132

    PubMed Central  CAS  PubMed  Google Scholar 

  50. Heutink P, van der Mey AG, Sandkuijl LA et al (1992) A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 11q23-qter. Hum Mol Genet 1:7–10

    CAS  PubMed  Google Scholar 

  51. van der Mey AG, Maaswinkel-Mooy PD, Cornelisse CJ, Schmidt PH, van de Kamp JJ (1989) Genomic imprinting in hereditary glomus tumours: evidence for new genetic theory. Lancet 2:1291–1294

    PubMed  Google Scholar 

  52. Hensen EF, Jordanova ES, van Minderhout IJ et al (2004) Somatic loss of maternal chromosome 11 causes parent-of-origin-dependent inheritance in SDHD-linked paraganglioma and phaeochromocytoma families. Oncogene 23:4076–4083

    CAS  PubMed  Google Scholar 

  53. Dahia PL, Ross KN, Wright ME et al (2005) A HIF1alpha regulatory loop links hypoxia and mitochondrial signals in pheochromocytomas. PLoS Genet 1:72–80

    CAS  PubMed  Google Scholar 

  54. Favier J, Briere JJ, Burnichon N et al (2009) The Warburg effect is genetically determined in inherited pheochromocytomas. PLoS One 4:e7094

    PubMed Central  PubMed  Google Scholar 

  55. Favier J, Gimenez-Roqueplo AP (2010) Pheochromocytomas: the (pseudo)-hypoxia hypothesis. Best Pract Res Clin Endocrinol Metab 24:957–968

    CAS  PubMed  Google Scholar 

  56. Qin Y, Buddavarapu K, Dahia PL (2009) Pheochromocytomas: from genetic diversity to new paradigms. Horm Metab Res 41:664–671

    CAS  PubMed  Google Scholar 

  57. Selak MA, Armour SM, MacKenzie ED et al (2005) Succinate links TCA cycle dysfunction to oncogenesis by inhibiting HIF-alpha prolyl hydroxylase. Cancer Cell 7:77–85

    CAS  PubMed  Google Scholar 

  58. Califano D, Rizzo C, D’Alessio A et al (2000) Signaling through Ras is essential for ret oncogene-induced cell differentiation in PC12 cells. J Biol Chem 275:19297–19305

    CAS  PubMed  Google Scholar 

  59. Johannessen CM, Reczek EE, James MF, Brems H, Legius E, Cichowski K (2005) The NF1 tumor suppressor critically regulates TSC2 and mTOR. Proc Natl Acad Sci U S A 102:8573–8578

    PubMed Central  CAS  PubMed  Google Scholar 

  60. Martin GA, Viskochil D, Bollag G et al (1990) The GAP-related domain of the neurofibromatosis type 1 gene product interacts with ras p21. Cell 63:843–849

    CAS  PubMed  Google Scholar 

  61. Johannessen CM, Johnson BW, Williams SM et al (2008) TORC1 is essential for NF1-associated malignancies. Curr Biol 18:56–62

    CAS  PubMed  Google Scholar 

  62. Lee S, Nakamura E, Yang H et al (2005) Neuronal apoptosis linked to EglN3 prolyl hydroxylase and familial pheochromocytoma genes: developmental culling and cancer. Cancer Cell 8:155–167

    PubMed  Google Scholar 

  63. Schlisio S, Kenchappa RS, Vredeveld LC et al (2008) The kinesin KIF1Bbeta acts downstream from EglN3 to induce apoptosis and is a potential 1p36 tumor suppressor. Genes Dev 22:884–893

    PubMed Central  CAS  PubMed  Google Scholar 

  64. Nagatsu T, Levitt M, Udenfriend S (1964) Tyrosine hydroxylase. The initial step in norepinephrine biosynthesis. J Biol Chem 239:2910–2917

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  66. Lenders JW, Eisenhofer G, Armando I, Keiser HR, Goldstein DS, Kopin IJ (1993) Determination of metanephrines in plasma by liquid chromatography with electrochemical detection. Clin Chem 39:97–103

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  68. 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

    CAS  PubMed  Google Scholar 

  69. Pacak K (2007) Preoperative management of the pheochromocytoma patient. J Clin Endocrinol Metab 92:4069–4079

    CAS  PubMed  Google Scholar 

  70. Brouwers FM, Eisenhofer G, Tao JJ et al (2006) High frequency of SDHB germline mutations in patients with malignant catecholamine-producing paragangliomas: implications for genetic testing. J Clin Endocrinol Metab 91:4505–4509

    CAS  PubMed  Google Scholar 

  71. van der Harst E, de Herder WW, de Krijger RR et al (2002) The value of plasma markers for the clinical behaviour of phaeochromocytomas. Eur J Endocrinol 147:85–94

    PubMed  Google Scholar 

  72. Montesinos MS, Machado JD, Camacho M et al (2008) The crucial role of chromogranins in storage and exocytosis revealed using chromaffin cells from chromogranin A null mouse. J Neurosci 28:3350–3358

    CAS  PubMed  Google Scholar 

  73. Guignat L, Bidart JM, Nocera M, Comoy E, Schlumberger M, Baudin E (2001) Chromogranin A and the alpha-subunit of glycoprotein hormones in medullary thyroid carcinoma and phaeochromocytoma. Br J Cancer 84:808–812

    PubMed Central  CAS  PubMed  Google Scholar 

  74. d’Herbomez M, Gouze V, Huglo D et al (2001) Chromogranin A assay and (131)I-MIBG scintigraphy for diagnosis and follow-up of pheochromocytoma. J Nucl Med 42:993–997

    PubMed  Google Scholar 

  75. Algeciras-Schimnich A, Preissner CM, Young WF Jr, Singh RJ, Grebe SK (2008) Plasma chromogranin A or urine fractionated metanephrines follow-up testing improves the diagnostic accuracy of plasma fractionated metanephrines for pheochromocytoma. J Clin Endocrinol Metab 93:91–95

    PubMed Central  CAS  PubMed  Google Scholar 

  76. Rao F, Keiser HR, O’Connor DT (2000) Malignant pheochromocytoma. Chromaffin granule transmitters and response to treatment. Hypertension 36:1045–1052

    CAS  PubMed  Google Scholar 

  77. Ilias I, Pacak K (2004) Current approaches and recommended algorithm for the diagnostic localization of pheochromocytoma. J Clin Endocrinol Metab 89:479–491

    CAS  PubMed  Google Scholar 

  78. Maurea S, Cuocolo A, Reynolds JC, Neumann RD, Salvatore M (1996) Diagnostic imaging in patients with paragangliomas. Computed tomography, magnetic resonance and MIBG scintigraphy comparison. Q J Nucl Med 40:365–371

    CAS  PubMed  Google Scholar 

  79. Sohaib SAA, Bomanji J, Evanson J, Reznek RH (2001) Imaging of the endocrine system. In: Grainger R, Allison D, Adam A, Dixon A (eds) Diagnostic radiology: a textbook of medical imaging, 4th edn. Churchill Livingston, London, pp 1367–1399

    Google Scholar 

  80. Fink IJ, Reinig JW, Dwyer AJ, Doppman JL, Linehan WM, Keiser HR (1985) MR imaging of pheochromocytomas. J Comput Assist Tomogr 9:454–458

    CAS  PubMed  Google Scholar 

  81. van den Berg R, Verbist BM, Mertens BJ, van der Mey AG, van Buchem MA (2004) Head and neck paragangliomas: improved tumor detection using contrast-enhanced 3D time-of-flight MR angiography as compared with fat-suppressed MR imaging techniques. AJNR Am J Neuroradiol 25:863–870

    PubMed  Google Scholar 

  82. Olsen WL, Dillon WP, Kelly WM, Norman D, Brant-Zawadzki M, Newton TH (1987) MR imaging of paragangliomas. AJR Am J Roentgenol 148:201–204

    CAS  PubMed  Google Scholar 

  83. Gritzmann N, Herold C, Haller J, Karnel F, Schwaighofer B (1987) Duplex sonography of tumors of the carotid body. Cardiovasc Intervent Radiol 10:280–284

    CAS  PubMed  Google Scholar 

  84. Shulkin BL, Ilias I, Sisson JC, Pacak K (2006) Current trends in functional imaging of pheochromocytomas and paragangliomas. Ann N Y Acad Sci 1073:374–382

    PubMed  Google Scholar 

  85. Nielsen JT, Nielsen BV, Rehling M (1996) Location of adrenal medullary pheochromocytoma by I-123 metaiodobenzylguanidine SPECT. Clin Nucl Med 21:695–699

    CAS  PubMed  Google Scholar 

  86. van der Harst E, de Herder WW, Bruining HA et al (2001) [(123)I]metaiodobenzylguanidine and [(111)In]octreotide uptake in benign and malignant pheochromocytomas. J Clin Endocrinol Metab 86:685–693

    PubMed  Google Scholar 

  87. Bustillo A, Telischi F, Weed D et al (2004) Octreotide scintigraphy in the head and neck. Laryngoscope 114:434–440

    CAS  PubMed  Google Scholar 

  88. Duet M, Sauvaget E, Petelle B et al (2003) Clinical impact of somatostatin receptor scintigraphy in the management of paragangliomas of the head and neck. J Nucl Med 44:1767–1774

    PubMed  Google Scholar 

  89. Myssiorek D, Tronco G (2005) 111Indium pentetreotide imaging in the evaluation of head and neck tumors. Laryngoscope 115:1707–1716

    PubMed  Google Scholar 

  90. Hoegerle S, Nitzsche E, Altehoefer C et al (2002) Pheochromocytomas: detection with 18F DOPA whole body PET–initial results. Radiology 222:507–512

    PubMed  Google Scholar 

  91. Timmers HJ, Kozupa A, Chen CC et al (2007) Superiority of fluorodeoxyglucose positron emission tomography to other functional imaging techniques in the evaluation of metastatic SDHB-associated pheochromocytoma and paraganglioma. J Clin Oncol 25:2262–2269

    PubMed  Google Scholar 

  92. Timmers HJ, Chen CC, Carrasquillo JA et al (2009) Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 94:4757–4767

    PubMed Central  CAS  PubMed  Google Scholar 

  93. Janetschek G, Finkenstedt G, Gasser R et al (1998) Laparoscopic surgery for pheochromocytoma: adrenalectomy, partial resection, excision of paragangliomas. J Urol 160:330–334

    CAS  PubMed  Google Scholar 

  94. Niemann U, Hiller W, Behrend M (2002) 25 years experience of the surgical treatment of phaeochromocytoma. Eur J Surg 168:716–719

    CAS  PubMed  Google Scholar 

  95. Diner EK, Franks ME, Behari A, Linehan WM, Walther MM (2005) Partial adrenalectomy: the National Cancer Institute experience. Urology 66:19–23

    PubMed  Google Scholar 

  96. Buhl T, Mortensen J, Kjaer A (2002) I-123 MIBG imaging and intraoperative localization of metastatic pheochromocytoma: a case report. Clin Nucl Med 27:183–185

    PubMed  Google Scholar 

  97. van Hulsteijn LT, Corssmit EP, van der Hiel B, Smit JW, Stokkel MP (2012) Is there a role for radioguided surgery with iodine-labeled metaiodobenzylguanidine in resection of neuroendocrine tumors? Clin Nucl Med 37:1083–1088

    PubMed  Google Scholar 

  98. Mukherjee JJ, Kaltsas GA, Islam N et al (2001) Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with (131)I-meta-iodobenzylguanidine [(131)I-mIBG]. Clin Endocrinol (Oxf) 55:47–60

    CAS  Google Scholar 

  99. van Houtum WH, Corssmit EP, Douwes Dekker PB et al (2005) Increased prevalence of catecholamine excess and phaeochromocytomas in a well-defined Dutch population with SDHD-linked head and neck paragangliomas. Eur J Endocrinol 152:87–94

    PubMed  Google Scholar 

  100. Lim JY, Kim J, Kim SH et al (2010) Surgical treatment of carotid body paragangliomas: outcomes and complications according to the shamblin classification. Clin Exp Otorhinolaryngol 3:91–95

    PubMed Central  PubMed  Google Scholar 

  101. Anand VK, Alemar GO, Sanders TS (1995) Management of the internal carotid artery during carotid body tumor surgery. Laryngoscope 105:231–235

    CAS  PubMed  Google Scholar 

  102. Bradshaw JW, Jansen JC (2005) Management of vagal paraganglioma: is operative resection really the best option? Surgery 137:225–228

    PubMed  Google Scholar 

  103. Luna-Ortiz K, Rascon-Ortiz M, Villavicencio-Valencia V, Granados-Garcia M, Herrera-Gomez A (2005) Carotid body tumors: review of a 20-year experience. Oral Oncol 41:56–61

    PubMed  Google Scholar 

  104. Wang SJ, Wang MB, Barauskas TM, Calcaterra TC (2000) Surgical management of carotid body tumors. Otolaryngol Head Neck Surg 123:202–206

    CAS  PubMed  Google Scholar 

  105. Hawthorne MR, Makek MS, Harris JP, Fisch U (1988) The histopathological and clinical features of irradiated and nonirradiated temporal paragangliomas. Laryngoscope 98:325–331

    CAS  PubMed  Google Scholar 

  106. Boedeker CC, Ridder GJ, Schipper J (2005) Paragangliomas of the head and neck: diagnosis and treatment. Fam Cancer 4:55–59

    CAS  PubMed  Google Scholar 

  107. Persky MS, Setton A, Niimi Y, Hartman J, Frank D, Berenstein A (2002) Combined endovascular and surgical treatment of head and neck paragangliomas–a team approach. Head Neck 24:423–431

    PubMed  Google Scholar 

  108. Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB (2007) The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer 14:569–585

    CAS  PubMed  Google Scholar 

  109. Lee JH, Barich F, Karnell LH et al (2002) National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 94:730–737

    PubMed  Google Scholar 

  110. Boedeker CC, Neumann HP, Maier W, Bausch B, Schipper J, Ridder GJ (2007) Malignant head and neck paragangliomas in SDHB mutation carriers. Otolaryngol Head Neck Surg 137:126–129

    PubMed  Google Scholar 

  111. Burnichon N, Rohmer V, Amar L et al (2009) The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. J Clin Endocrinol Metab 94:2817–2827

    CAS  PubMed  Google Scholar 

  112. Neumann HP, Pawlu C, Peczkowska M et al (2004) Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA 292:943–951

    CAS  PubMed  Google Scholar 

  113. Benn DE, Gimenez-Roqueplo AP, Reilly JR et al (2006) Clinical presentation and penetrance of pheochromocytoma/paraganglioma syndromes. J Clin Endocrinol Metab 91:827–836

    CAS  PubMed  Google Scholar 

  114. Havekes B, Corssmit EP, Jansen JC, van der Mey AG, Vriends AH, Romijn JA (2007) Malignant paragangliomas associated with mutations in the succinate dehydrogenase D gene. J Clin Endocrinol Metab 92:1245–1248

    CAS  PubMed  Google Scholar 

  115. Pacak K, Ilias I, Adams KT, Eisenhofer G (2005) Biochemical diagnosis, localization and management of pheochromocytoma: focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumour. J Intern Med 257:60–68

    CAS  PubMed  Google Scholar 

  116. Loh KC, Fitzgerald PA, Matthay KK, Yeo PP, Price DC (1997) The treatment of malignant pheochromocytoma with iodine-131 metaiodobenzylguanidine (131I-MIBG): a comprehensive review of 116 reported patients. J Endocrinol Invest 20:648–658

    CAS  PubMed  Google Scholar 

  117. Huang H, Abraham J, Hung E et al (2008) Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer 113:2020–2028

    CAS  PubMed  Google Scholar 

  118. Keiser HR, Goldstein DS, Wade JL, Douglas FL, Averbuch SD (1985) Treatment of malignant pheochromocytoma with combination chemotherapy. Hypertension 7:I18–I24

    CAS  PubMed  Google Scholar 

  119. Forrer F, Riedweg I, Maecke HR, Mueller-Brand J (2008) Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma. Q J Nucl Med Mol Imaging 52:334–340

    CAS  PubMed  Google Scholar 

  120. Joshua AM, Ezzat S, Asa SL et al (2009) Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma. J Clin Endocrinol Metab 94:5–9

    CAS  PubMed  Google Scholar 

  121. Maithel SK, Fong Y (2009) Hepatic ablation for neuroendocrine tumor metastases. J Surg Oncol 100:635–638

    PubMed  Google Scholar 

  122. Takahashi K, Ashizawa N, Minami T et al (1999) Malignant pheochromocytoma with multiple hepatic metastases treated by chemotherapy and transcatheter arterial embolization. Intern Med 38:349–354

    CAS  PubMed  Google Scholar 

  123. Scholz T, Eisenhofer G, Pacak K, Dralle H, Lehnert H (2007) Clinical review: current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab 92:1217–1225

    CAS  PubMed  Google Scholar 

  124. Edstrom EE, Hjelm Skog AL, Hoog A, Hamberger B (2003) The management of benign and malignant pheochromocytoma and abdominal paraganglioma. Eur J Surg Oncol 29:278–283

    Google Scholar 

  125. van Gils AP, van der Mey AG, Hoogma RP et al (1992) MRI screening of kindred at risk of developing paragangliomas: support for genomic imprinting in hereditary glomus tumours. Br J Cancer 65:903–907

    PubMed Central  PubMed  Google Scholar 

  126. Hermsen MA, Sevilla MA, Llorente JL et al (2010) Relevance of germline mutation screening in both familial and sporadic head and neck paraganglioma for early diagnosis and clinical management. Cell Oncol 32:275–283

    CAS  PubMed  Google Scholar 

  127. 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 163:121–127

    PubMed  Google Scholar 

  128. Gill AJ, Benn DE, Chou A et al (2010) Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes. Hum Pathol 41:805–814

    CAS  PubMed  Google Scholar 

  129. van Nederveen FH, Gaal J, Favier J et al (2009) An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis. Lancet Oncol 10:764–771

    PubMed  Google Scholar 

  130. Timmers HJ, Gimenez-Roqueplo AP, Mannelli M, Pacak K (2009) Clinical aspects of SDHx-related pheochromocytoma and paraganglioma. Endocr Relat Cancer 16:391–400

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Endocrinology, Leiden University Medical Center, 9600, Leiden, 2300 RC, The Netherlands

    Eleonora P. Corssmit & Leonie T. van Hulsteijn

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  1. Eleonora P. Corssmit
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  2. Leonie T. van Hulsteijn
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Correspondence to Eleonora P. Corssmit .

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  1. Department of Medical Oncology, Hacettepe University Cancer Institute, Ankara, Turkey

    Suayib Yalcin

  2. Department of Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden

    Kjell Öberg

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Corssmit, E.P., van Hulsteijn, L.T. (2015). Pheochromocytoma and Paraganglioma. In: Yalcin, S., Öberg, K. (eds) Neuroendocrine Tumours. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45215-8_24

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