Skip to main content
SpringerLink
Log in

Management of Pheochromocytoma and Paraganglioma

  • Chapter
  • First Online:
Management of Adrenal Masses in Children and Adults

  • 814 Accesses

Abstract

Pheochromocytomas and paragangliomas are rare catecholamine-secreting neuroendocrine tumors, up to 40 % of which are attributed to germ line mutations in 12-well identified genes. Patients present with a wide range of clinical manifestations including paroxysms of hypertension, headaches, palpitations, and sweating, being the most common. The high levels of circulating catecholamines predispose patients to catastrophic cardiovascular complications, necessitating a prompt diagnosis and treatment intervention. The mainstay of diagnosis is based on biochemical evidence, which should be followed by localization of the tumor. In addition, genetic screening has also become a standard diagnostic modality and is particularly important in patients with an earlier onset and multifocal disease. Patients should receive symptomatic management and stabilization of the hemodynamic status, which can be achieved with alpha and beta adrenoceptor blockade, calcium channel blockers, and inhibitors of catecholamine synthesis. Surgical resection represents the main form of curative treatment in patients with nonmetastatic disease. Optimization of the preoperative hemodynamic status is critical in preventing intraoperative and postoperative instability and complications. For patients with metastatic disease, other treatment modalities including chemotherapy, radiofrequency ablation, external beam radiation, and peptide receptor radionuclide therapy with MIBG or DOTA peptides should be considered. These treatment modalities may be used alone or in synergistic combinations. Furthermore, for refractory cases, novel treatments including targeted molecular therapies should be considered.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. McNeil AR, Blok BH, Koelmeyer TD, Burke MP, Hilton JM. Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland. Aust N Z J Med. 2000;30(6):648–52.

    Article  CAS  PubMed  Google Scholar 

  2. Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet. 2005;366(9486):665–75.

    Article  PubMed  Google Scholar 

  3. Pacak K, Linehan WM, Eisenhofer G, Walther MM, Goldstein DS. Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma. Ann Intern Med. 2001;134(4):315–29.

    Article  CAS  PubMed  Google Scholar 

  4. Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol. 1992;147(1):1–10.

    CAS  PubMed  Google Scholar 

  5. Baysal BE, Myers EN. Etiopathogenesis and clinical presentation of carotid body tumors. Microsc Res Tech. 2002;59(3):256–61.

    Article  PubMed  Google Scholar 

  6. Erickson D, Kudva YC, Ebersold MJ, Thompson GB, Grant CS, van Heerden JA, et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab. 2001;86(11):5210–6.

    Article  CAS  PubMed  Google Scholar 

  7. Taieb D, Kaliski A, Boedeker CC, Martucci V, Fojo T, Adler Jr JR, et al. Current approaches and recent developments in the management of head and neck paragangliomas. Endocr Rev. 2014;35(5):795–819.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Eisenhofer G, Peitzsch M. Laboratory evaluation of pheochromocytoma and paraganglioma. Clin Chem. 2014;60(12):1486–99.

    Article  CAS  PubMed  Google Scholar 

  9. Eisenhofer G, Lenders JW, Goldstein DS, Mannelli M, Csako G, Walther MM, et al. Pheochromocytoma catecholamine phenotypes and prediction of tumor size and location by use of plasma free metanephrines. Clin Chem. 2005;51(4):735–44.

    Article  CAS  PubMed  Google Scholar 

  10. Van Der Horst-Schrivers AN, Osinga TE, Kema IP, Van Der Laan BF, Dullaart RP. Dopamine excess in patients with head and neck paragangliomas. Anticancer Res. 2010;30(12):5153–8.

    Google Scholar 

  11. van der Harst E, de Herder WW, de Krijger RR, Bruining HA, Bonjer HJ, Lamberts SW, et al. The value of plasma markers for the clinical behaviour of phaeochromocytomas. Eur J Endocrinol. 2002;147(1):85–94.

    Article  PubMed  Google Scholar 

  12. Favier J, Amar L, Gimenez-Roqueplo AP. Paraganglioma and phaeochromocytoma: from genetics to personalized medicine. Nat Rev Endocrinol. 2015;11(2):101–11.

    Article  CAS  PubMed  Google Scholar 

  13. Gimenez-Roqueplo AP, Dahia PL, Robledo M. An update on the genetics of paraganglioma, pheochromocytoma, and associated hereditary syndromes. Horm Metab Res. 2012;44(5):328–33.

    Article  CAS  PubMed  Google Scholar 

  14. Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014;38(1):7–41.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Jochmanova I, Yang C, Zhuang Z, Pacak K. Hypoxia-inducible factor signaling in pheochromocytoma: turning the rudder in the right direction. J Natl Cancer Inst. 2013;105(17):1270–83.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Eisenhofer G, Huynh TT, Pacak K, Brouwers FM, Walther MM, Linehan WM, et al. 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. 2004;11(4):897–911.

    Article  CAS  PubMed  Google Scholar 

  17. Pacak K. Preoperative management of the pheochromocytoma patient. J Clin Endocrinol Metab. 2007;92(11):4069–79.

    Article  CAS  PubMed  Google Scholar 

  18. Pacak K. Phaeochromocytoma: a catecholamine and oxidative stress disorder. Endocr Regul. 2011;45(2):65–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Pacak K, Lenders JWM, Eisenhofer G. Pheochromocytoma: diagnosis, localization, and treatment. Vol vi. Malden, MA: Blackwell Pub.; 2007. 172 p.

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  21. Ito Y, Fujimoto Y, Obara T. The role of epinephrine, norepinephrine, and dopamine in blood pressure disturbances in patients with pheochromocytoma. World J Surg. 1992;16(4):759–63. discussion 63-4.

    Article  CAS  PubMed  Google Scholar 

  22. Page LB, Raker JW, Berberich FR. Pheochromocytoma with predominant epinephrine secretion. Am J Med. 1969;47(4):648–52.

    Article  CAS  PubMed  Google Scholar 

  23. Ueda T, Oka N, Matsumoto A, Miyazaki H, Ohmura H, Kikuchi T, et al. Pheochromocytoma presenting as recurrent hypotension and syncope. Intern Med. 2005;44(3):222–7.

    Article  PubMed  Google Scholar 

  24. Zuber SM, Kantorovich V, Pacak K. Hypertension in pheochromocytoma: characteristics and treatment. Endocrinol Metab Clin North Am. 2011;40(2):295–311. vii.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Fujiwara M, Imachi H, Murao K, Muraoka T, Ohyama T, Miyai Y, et al. Improvement in renal dysfunction and symptoms after laparoscopic adrenalectomy in a patient with pheochromocytoma complicated by renal dysfunction. Endocrine. 2009;35(1):57–62.

    Article  CAS  PubMed  Google Scholar 

  26. Petkou D, Petropoulos IK, Kordelou A, Katsimpris JM. Severe bilateral hypertensive retinopathy and optic neuropathy in a patient with pheochromocytoma. Klin Monbl Augenheilkd. 2008;225(5):500–3.

    Article  CAS  PubMed  Google Scholar 

  27. Carr ND, Hulme A, Sheron N, Lees WR, Russell RC. Intestinal ischaemia associated with phaeochromocytoma. Postgrad Med J. 1989;65(766):594–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Oliver MD, Lipkin GW, Brownjohn AM. Intestinal ischaemia associated with phaeochromocytoma. Postgrad Med J. 1990;66(772):156.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Rosas AL, Kasperlik-Zaluska AA, Papierska L, Bass BL, Pacak K, Eisenhofer G. Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature. Eur J Endocrinol. 2008;158(3):423–9.

    Article  CAS  PubMed  Google Scholar 

  30. Proye C, Fossati P, Fontaine P, Lefebvre J, Decoulx M, Wemeau JL, et al. Dopamine-secreting pheochromocytoma: an unrecognized entity? Classification of pheochromocytomas according to their type of secretion. Surgery. 1986;100(6):1154–62.

    CAS  PubMed  Google Scholar 

  31. Gangopadhyay K, Baskar V, Toogood A. A case of exclusive dopamine-secreting paraganglioma. Clin Endocrinol (Oxf). 2008;68(3):494–5.

    CAS  Google Scholar 

  32. Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai EW, et al. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 2005;90(4):2068–75.

    Article  CAS  PubMed  Google Scholar 

  33. Dubois LA, Gray DK. Dopamine-secreting pheochromocytomas: in search of a syndrome. World J Surg. 2005;29(7):909–13.

    Article  PubMed  Google Scholar 

  34. Shen WW. Vomiting, chemoreceptor trigger zone, and dopamine. Psychosomatics. 1989;30(1):118–9.

    Article  CAS  PubMed  Google Scholar 

  35. Eisenhofer G, Bornstein SR, Brouwers FM, Cheung NK, Dahia PL, de Krijger RR, et al. Malignant pheochromocytoma: current status and initiatives for future progress. Endocr Relat Cancer. 2004;11(3):423–36.

    Article  CAS  PubMed  Google Scholar 

  36. Ayala-Ramirez M, Feng L, Johnson MM, Ejaz S, Habra MA, Rich T, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metabol. 2011;96(3):717–25.

    Article  CAS  Google Scholar 

  37. Bravo EL, Tagle R. Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev. 2003;24(4):539–53.

    Article  CAS  PubMed  Google Scholar 

  38. Strong VE, Kennedy T, Al-Ahmadie H, Tang L, Coleman J, Fong Y, et al. Prognostic indicators of malignancy in adrenal pheochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery. 2008;143(6):759–68.

    Article  PubMed  Google Scholar 

  39. Brown HM, Komorowski RA, Wilson SD, Demeure MJ, Zhu YR. Predicting metastasis of pheochromocytomas using DNA flow cytometry and immunohistochemical markers of cell proliferation: a positive correlation between MIB-1 staining and malignant tumor behavior. Cancer. 1999;86(8):1583–9.

    Article  CAS  PubMed  Google Scholar 

  40. Eisenhofer G, Lenders JW, Siegert G, Bornstein SR, Friberg P, Milosevic D, et al. 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. 2012;48(11):1739–49.

    Article  CAS  PubMed  Google Scholar 

  41. Eisenhofer G, Lenders JW, Timmers H, Mannelli M, Grebe SK, Hofbauer LC, et al. Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma. Clin Chem. 2011;57(3):411–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Stolk RF, Bakx C, Mulder J, Timmers HJ, Lenders JW. Is the excess cardiovascular morbidity in pheochromocytoma related to blood pressure or to catecholamines? J Clin Endocrinol Metab. 2013;98(3):1100–6.

    Article  CAS  PubMed  Google Scholar 

  43. Galetta F, Franzoni F, Bernini G, Poupak F, Carpi A, Cini G, et al. Cardiovascular complications in patients with pheochromocytoma: a mini-review. Biomed Pharmacother. 2010;64(7):505–9.

    Article  CAS  PubMed  Google Scholar 

  44. Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002;287(11):1427–34.

    Article  CAS  PubMed  Google Scholar 

  45. Boot C, Toole B, Johnson SJ, Ball SG, Neely D. ANNALS EXPRESS: single-centre study of the diagnostic performance of plasma metanephrines with seated sampling for the diagnosis of phaeochromocytoma/paraganglioma. Ann Clin Biochem. 2016.

    Google Scholar 

  46. Casey R, Griffin TP, Wall D, Dennedy MC, Bell M, O'Shea PM. Screening for phaeochromocytoma and paraganglioma: impact of using supine reference intervals for plasma metanephrines with samples collected from fasted/seated patients. Ann Clin Biochem. 2016.

    Google Scholar 

  47. Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K, et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010;39(6):775–83.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, et al. Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab. 2007;3(2):92–102.

    Article  PubMed  Google Scholar 

  49. Weismann D, Peitzsch M, Raida A, Prejbisz A, Gosk M, Riester A, et al. Measurements of plasma metanephrines by immunoassay vs liquid chromatography with tandem mass spectrometry for diagnosis of pheochromocytoma. Eur J Endocrinol. 2015;172(3):251–60.

    Article  CAS  PubMed  Google Scholar 

  50. van Berkel A, Lenders JW, Timmers HJ. Diagnosis of endocrine disease: biochemical diagnosis of phaeochromocytoma and paraganglioma. Eur J Endocrinol. 2014;170(3):R109–19.

    Article  PubMed  CAS  Google Scholar 

  51. Kimura N, Miura W, Noshiro T, Mizunashi K, Hanew K, Shimizu K, et al. Plasma chromogranin A in pheochromocytoma, primary hyperparathyroidism and pituitary adenoma in comparison with catecholamine, parathyroid hormone and pituitary hormones. Endocr J. 1997;44(2):319–27.

    Article  CAS  PubMed  Google Scholar 

  52. Kimura N, Sasano N, Yamada R, Satoh J. Immunohistochemical study of chromogranin in 100 cases of pheochromocytoma, carotid body tumour, medullary thyroid carcinoma and carcinoid tumour. Virchows Arch A Pathol Anat Histopathol. 1988;413(1):33–8.

    Article  CAS  PubMed  Google Scholar 

  53. Zuber S, Wesley R, Prodanov T, Eisenhofer G, Pacak K, Kantorovich V. Clinical utility of chromogranin A in SDHx-related paragangliomas. Eur J Clin Invest. 2014;44(4):365–71.

    Article  CAS  PubMed  Google Scholar 

  54. Eisenhofer G, Goldstein DS, Walther MM, Friberg P, Lenders JW, Keiser HR, et al. Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results. J Clin Endocrinol Metab. 2003;88(6):2656–66.

    Article  CAS  PubMed  Google Scholar 

  55. Eisenhofer G, Siegert G, Kotzerke J, Bornstein SR, Pacak K. Current progress and future challenges in the biochemical diagnosis and treatment of pheochromocytomas and paragangliomas. Horm Metab Res. 2008;40(5):329–37.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  56. Neary NM, King KS, Pacak K. Drugs and pheochromocytoma—don’t be fooled by every elevated metanephrine. N Engl J Med. 2011;364(23):2268–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915–42.

    Article  CAS  PubMed  Google Scholar 

  58. Ilias I, Pacak K. Current approaches and recommended algorithm for the diagnostic localization of pheochromocytoma. J Clin Endocrinol Metab. 2004;89(2):479–91.

    Article  CAS  PubMed  Google Scholar 

  59. Ilias I, Pacak K. Diagnosis, localization and treatment of pheochromocytoma in MEN 2 syndrome. Endocr Regul. 2009;43(2):89–93.

    CAS  PubMed  Google Scholar 

  60. Ilias I, Chen CC, Carrasquillo JA, Whatley M, Ling A, Lazurova I, et al. Comparison of 6-18F-fluorodopamine PET with 123I-metaiodobenzylguanidine and 111in-pentetreotide scintigraphy in localization of nonmetastatic and metastatic pheochromocytoma. J Nucl Med. 2008;49(10):1613–9.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Timmers HJ, Chen CC, Carrasquillo JA, Whatley M, Ling A, Eisenhofer G, et al. Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography. J Natl Cancer Inst. 2012;104(9):700–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Zelinka T, Timmers HJ, Kozupa A, Chen CC, Carrasquillo JA, Reynolds JC, et al. Role of positron emission tomography and bone scintigraphy in the evaluation of bone involvement in metastatic pheochromocytoma and paraganglioma: specific implications for succinate dehydrogenase enzyme subunit B gene mutations. Endocr Relat Cancer. 2008;15(1):311–23.

    Article  PubMed  Google Scholar 

  63. King KS, Chen CC, Alexopoulos DK, Whatley MA, Reynolds JC, Patronas N, et al. Functional imaging of SDHx-related head and neck paragangliomas: comparison of 18F-fluorodihydroxyphenylalanine, 18F-fluorodopamine, 18F-fluoro-2-deoxy-D-glucose PET, 123I-metaiodobenzylguanidine scintigraphy, and 111In-pentetreotide scintigraphy. J Clin Endocrinol Metab. 2011;96(9):2779–85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  64. Marzola MC, Chondrogiannis S, Grassetto G, Rampin L, Maffione AM, Ferretti A, et al. 18F-DOPA PET/CT in the evaluation of hereditary SDH-deficiency paraganglioma-pheochromocytoma syndromes. Clin Nucl Med. 2014;39(1):e53–8.

    Article  PubMed  Google Scholar 

  65. Miederer M, Fottner C, Rossmann H, Helisch A, Papaspyrou K, Bartsch O, et al. High incidence of extraadrenal paraganglioma in families with SDHx syndromes detected by functional imaging with [18F]fluorodihydroxyphenylalanine PET. Eur J Nucl Med Mol Imaging. 2013;40(6):889–96.

    Article  CAS  PubMed  Google Scholar 

  66. Gabriel S, Blanchet EM, Sebag F, Chen CC, Fakhry N, Deveze A, et al. Functional characterization of nonmetastatic paraganglioma and pheochromocytoma by (18) F-FDOPA PET: focus on missed lesions. Clin Endocrinol (Oxf). 2013;79(2):170–7.

    Article  CAS  Google Scholar 

  67. Janssen I, Chen CC, Millo CM, Ling A, Taieb D, Lin FI, et al. PET/CT comparing 68Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2016.

    Google Scholar 

  68. Maurice JB, Troke R, Win Z, Ramachandran R, Al-Nahhas A, Naji M, et al. 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. 2012;39(8):1266–70.

    Article  CAS  PubMed  Google Scholar 

  69. Tan TH, Hussein Z, Saad FF, Shuaib IL. Diagnostic performance of (68)Ga-DOTATATE PET/CT, (18)F-FDG PET/CT and (131)I-MIBG scintigraphy in mapping metastatic pheochromocytoma and paraganglioma. Nucl Med Mol Imaging. 2015;49(2):143–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  70. Janssen I, Blanchet EM, Adams K, Chen CC, Millo CM, Herscovitch P, et al. Superiority of [68Ga]-DOTATATE PET/CT to other functional imaging modalities in the localization of SDHB-associated metastatic pheochromocytoma and paraganglioma. Clin Cancer Res. 2015;21(17):3888–95.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  71. Janssen I, Chen CC, Taieb D, Patronas NJ, Millo CM, Adams KT, et al. 68Ga-DOTATATE PET/CT in the localization of head and neck paragangliomas compared with other functional imaging modalities and CT/MRI. J Nucl Med. 2016;57(2):186–91.

    Article  PubMed  Google Scholar 

  72. Wang W, Li P, Wang Y, Wang Y, Ma Z, Wang G, et al. Effectiveness and safety of laparoscopic adrenalectomy of large pheochromocytoma: a prospective, nonrandomized, controlled study. Am J Surg. 2015;210(2):230–5.

    Article  PubMed  Google Scholar 

  73. Conzo G, Tartaglia E, Gambardella C, Esposito D, Sciascia V, Mauriello C, et al. Minimally invasive approach for adrenal lesions: systematic review of laparoscopic versus retroperitoneoscopic adrenalectomy and assessment of risk factors for complications. Int J Surg. 2016;28 Suppl 1:S118–23.

    Article  PubMed  Google Scholar 

  74. Conzo G, Musella M, Corcione F, De Palma M, Ferraro F, Palazzo A, et al. Laparoscopic adrenalectomy, a safe procedure for pheochromocytoma. A retrospective review of clinical series. Int J Surg. 2013;11(2):152–6.

    Article  PubMed  Google Scholar 

  75. Walz MK, Alesina PF, Wenger FA, Deligiannis A, Szuczik E, Petersenn S, et al. Posterior retroperitoneoscopic adrenalectomy—results of 560 procedures in 520 patients. Surgery. 2006;140(6):943–8. discussion 8-50.

    Article  PubMed  Google Scholar 

  76. Alesina PF, Hommeltenberg S, Meier B, Petersenn S, Lahner H, Schmid KW, et al. Posterior retroperitoneoscopic adrenalectomy for clinical and subclinical Cushing’s syndrome. World J Surg. 2010;34(6):1391–7.

    Article  PubMed  Google Scholar 

  77. Shen WT, Grogan R, Vriens M, Clark OH, Duh QY. One hundred two patients with pheochromocytoma treated at a single institution since the introduction of laparoscopic adrenalectomy. Arch Surg. 2010;145(9):893–7.

    Article  PubMed  Google Scholar 

  78. Walz MK, Groeben H, Alesina PF. Single-access retroperitoneoscopic adrenalectomy (SARA) versus conventional retroperitoneoscopic adrenalectomy (CORA): a case-control study. World J Surg. 2010;34(6):1386–90.

    Article  PubMed  Google Scholar 

  79. Goers TA, Abdo M, Moley JF, Matthews BD, Quasebarth M, Brunt LM. Outcomes of resection of extra-adrenal pheochromocytomas/paragangliomas in the laparoscopic era: a comparison with adrenal pheochromocytoma. Surg Endosc. 2013;27(2):428–33.

    Article  PubMed  Google Scholar 

  80. Kim HH, Kim GH, Sung GT. Laparoscopic adrenalectomy for pheochromocytoma: comparison with conventional open adrenalectomy. J Endourol. 2004;18(3):251–5.

    Article  CAS  PubMed  Google Scholar 

  81. Conzo G, Musella M, Corcione F, De Palma M, Avenia N, Milone M, et al. Laparoscopic treatment of pheochromocytomas smaller or larger than 6 cm. A clinical retrospective study on 44 patients. Laparoscopic adrenalectomy for pheochromocytoma. Ann Ital Chir. 2013;84(4):417–22.

    PubMed  Google Scholar 

  82. Carter YM, Mazeh H, Sippel RS, Chen H. Safety and feasibility of laparoscopic resection for large (>/= 6 CM) pheochromocytomas without suspected malignancy. Endocr Pract. 2012;18(5):720–6.

    Article  PubMed  PubMed Central  Google Scholar 

  83. Aliyev S, Karabulut K, Agcaoglu O, Wolf K, Mitchell J, Siperstein A, et al. Robotic versus laparoscopic adrenalectomy for pheochromocytoma. Ann Surg Oncol. 2013;20(13):4190–4.

    Article  PubMed  Google Scholar 

  84. Arghami A, Dy BM, Bingener J, Osborn J, Richards ML. Single-port robotic-assisted adrenalectomy: feasibility, safety, and cost-effectiveness. JSLS. 2015;19(1), e2014.00218.

    Article  PubMed  PubMed Central  Google Scholar 

  85. Asher KP, Gupta GN, Boris RS, Pinto PA, Linehan WM, Bratslavsky G. Robot-assisted laparoscopic partial adrenalectomy for pheochromocytoma: the National Cancer Institute technique. Eur Urol. 2011;60(1):118–24.

    Article  PubMed  PubMed Central  Google Scholar 

  86. Benhammou JN, Boris RS, Pacak K, Pinto PA, Linehan WM, Bratslavsky G. 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. 2010;184(5):1855–9.

    Article  PubMed  PubMed Central  Google Scholar 

  87. Castinetti F, Taieb D, Henry JF, Walz M, Guerin C, Brue T, et al. Management of endocrine disease: outcome of adrenal sparing surgery in heritable pheochromocytoma. Eur J Endocrinol. 2016;174(1):R9–18.

    CAS  PubMed  Google Scholar 

  88. Grubbs EG, Rich TA, Ng C, Bhosale PR, Jimenez C, Evans DB, et al. Long-term outcomes of surgical treatment for hereditary pheochromocytoma. J Am Coll Surg. 2013;216(2):280–9.

    Article  PubMed  Google Scholar 

  89. Alesina PF, Hinrichs J, Meier B, Schmid KW, Neumann HP, Walz MK. Minimally invasive cortical-sparing surgery for bilateral pheochromocytomas. Langenbecks Arch Surg. 2012;397(2):233–8.

    Article  PubMed  Google Scholar 

  90. Lee JE, Curley SA, Gagel RF, Evans DB, Hickey RC. Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma. Surgery. 1996;120(6):1064–70. discussion 70-1.

    Article  CAS  PubMed  Google Scholar 

  91. Neumann HP, Reincke M, Bender BU, Elsner R, Janetschek G. Preserved adrenocortical function after laparoscopic bilateral adrenal sparing surgery for hereditary pheochromocytoma. J Clin Endocrinol Metab. 1999;84(8):2608–10.

    CAS  PubMed  Google Scholar 

  92. Nambirajan T, Bagheri F, Abdelmaksoud A, Leeb K, Neumann H, Graubner UB, et al. Laparoscopic partial adrenalectomy for recurrent pheochromocytoma in a boy with Von Hippel-Lindau disease. J Laparoendosc Adv Surg Tech A. 2004;14(4):234–5.

    Article  PubMed  Google Scholar 

  93. Ellis RJ, Patel D, Prodanov T, Sadowski S, Nilubol N, Adams K, et al. Response after surgical resection of metastatic pheochromocytoma and paraganglioma: can postoperative biochemical remission be predicted? J Am Coll Surg. 2013;217(3):489–96.

    Article  PubMed  PubMed Central  Google Scholar 

  94. Arnas-Leon C, Sanchez V, Santana Suarez AD, Quintana Arroyo S, Acosta C, Martinez Martin FJ. Complete remission in metastatic pheochromocytoma treated with extensive surgery. Cureus. 2016;8(1), e447.

    PubMed  PubMed Central  Google Scholar 

  95. Una-Gorospe JA, Munoz-Iglesias J, De Sequera-Rahola M, Anton L. Usefulness of single photon emission computed tomography (SPECT)/computed tomography and radioguided surgery in a patient with recurrent pheochromocytoma. Indian J Nucl Med. 2013;28(1):59–60.

    Article  PubMed  PubMed Central  Google Scholar 

  96. Buhl T, Mortensen J, Kjaer A. I-123 MIBG imaging and intraoperative localization of metastatic pheochromocytoma: a case report. Clin Nucl Med. 2002;27(3):183–5.

    Article  PubMed  Google Scholar 

  97. Sadowski SM, Millo C, Neychev V, Aufforth R, Keutgen X, Glanville J, et al. Feasibility of radio-guided surgery with (6)(8)Gallium-DOTATATE in patients with gastro-entero-pancreatic neuroendocrine tumors. Ann Surg Oncol. 2015;22 Suppl 3:S676–82.

    Article  PubMed  Google Scholar 

  98. Adjalle R, Plouin PF, Pacak K, Lehnert H. Treatment of malignant pheochromocytoma. Horm Metab Res. 2009;41(9):687–96.

    Article  CAS  PubMed  Google Scholar 

  99. Prys-Roberts C. Phaeochromocytoma—recent progress in its management. Br J Anaesth. 2000;85(1):44–57.

    Article  CAS  PubMed  Google Scholar 

  100. Azadeh N, Ramakrishna H, Bhatia NL, Charles JC, Mookadam F. Therapeutic goals in patients with pheochromocytoma: a guide to perioperative management. Ir J Med Sci. 2016;185(1):43–9.

    Article  CAS  PubMed  Google Scholar 

  101. Meune C, Bertherat J, Dousset B, Jude N, Bertagna X, Duboc D, et al. Reduced myocardial contractility assessed by tissue Doppler echocardiography is associated with increased risk during adrenal surgery of patients with pheochromocytoma: report of a preliminary study. J Am Soc Echocardiogr. 2006;19(12):1466–70.

    Article  PubMed  Google Scholar 

  102. Goldstein RE, O'Neill Jr JA, Holcomb 3rd GW, Morgan 3rd WM, Neblett 3rd WW, Oates JA, et al. Clinical experience over 48 years with pheochromocytoma. Ann Surg. 1999;229(6):755–64. discussion 64-6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  103. Li J, Yang CH. Improvement of preoperative management in patients with adrenal pheochromocytoma. Int J Clin Exp Med. 2014;7(12):5541–6.

    PubMed  PubMed Central  Google Scholar 

  104. Prys-Roberts C, Farndon JR. Efficacy and safety of doxazosin for perioperative management of patients with pheochromocytoma. World J Surg. 2002;26(8):1037–42.

    Article  PubMed  Google Scholar 

  105. Mallat J, Pironkov A, Destandau MS, Tavernier B. Systolic pressure variation (Deltadown) can guide fluid therapy during pheochromocytoma surgery. Can J Anaesth. 2003;50(10):998–1003.

    Article  PubMed  Google Scholar 

  106. Kinney MA, Narr BJ, Warner MA. Perioperative management of pheochromocytoma. J Cardiothorac Vasc Anesth. 2002;16(3):359–69.

    Article  PubMed  Google Scholar 

  107. Ahmed A. Perioperative management of pheochromocytoma: anaesthetic implications. J Pak Med Assoc. 2007;57(3):140–6.

    PubMed  Google Scholar 

  108. Kutikov A, Cripsen P, Uzzo R. Pathophysiology, evaluation and medical management of adrenal disorders. In: Wein A, Kavoussi L, Novick A, Partin A, Peters C, editors. Campbell-Walsh urology, vol. 2. 10th ed. Philadelphia, PA: Elsevier; 2012. p. 1685–736.

    Chapter  Google Scholar 

  109. Perry RR, Keiser HR, Norton JA, Wall RT, Robertson CN, Travis W, et al. Surgical management of pheochromocytoma with the use of metyrosine. Ann Surg. 1990;212(5):621–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  110. Steinsapir J, Carr AA, Prisant LM, Bransome Jr ED. Metyrosine and pheochromocytoma. Arch Intern Med. 1997;157(8):901–6.

    Article  CAS  PubMed  Google Scholar 

  111. Robinson RG, DeQuattro V, Grushkin CM, Lieberman E. Childhood pheochromocytoma: treatment with alpha methyl tyrosine for resistant hypertension. J Pediatr. 1977;91(1):143–7.

    Article  CAS  PubMed  Google Scholar 

  112. Wachtel H, Kennedy EH, Zaheer S, Bartlett EK, Fishbein L, Roses RE, et al. Preoperative metyrosine improves cardiovascular outcomes for patients undergoing surgery for pheochromocytoma and paraganglioma. Ann Surg Oncol. 2015;22 Suppl 3:S646–54.

    Article  PubMed  Google Scholar 

  113. Brogden RN, Heel RC, Speight TM, Avery GS. α-Methyl-p-tyrosine: a review of its pharmacology and clinical use. Drugs. 1981;21(2):81–9.

    Article  CAS  PubMed  Google Scholar 

  114. Young Jr WF. Pheochromocytoma: issues in diagnosis & treatment. Compr Ther. 1997;23(5):319–26.

    PubMed  Google Scholar 

  115. Young JL. Catecholamines and the adrenal medulla. In: Wilson JF, Kronenburg HM, Larsen PR, editors. Williams textbook of endocrinology. Philadelphia, PA: WB Saunder Co.; 1998. p. 665–728.

    Google Scholar 

  116. Jovenich JJ. Anesthesia in adrenal surgery. Urol Clin North Am. 1989;16(3):583–7.

    CAS  PubMed  Google Scholar 

  117. Hull CJ. Phaeochromocytoma. Diagnosis, preoperative preparation and anaesthetic management. Br J Anaesth. 1986;58(12):1453–68.

    Article  CAS  PubMed  Google Scholar 

  118. Desmonts JM, Marty J. Anaesthetic management of patients with phaeochromocytoma. Br J Anaesth. 1984;56(7):781–9.

    Article  CAS  PubMed  Google Scholar 

  119. James MF. Use of magnesium sulphate in the anaesthetic management of phaeochromocytoma: a review of 17 anaesthetics. Br J Anaesth. 1989;62(6):616–23.

    Article  CAS  PubMed  Google Scholar 

  120. Proye C, Thevenin D, Cecat P, Petillot P, Carnaille B, Verin P, et al. Exclusive use of calcium channel blockers in preoperative and intraoperative control of pheochromocytomas: hemodynamics and free catecholamine assays in ten consecutive patients. Surgery. 1989;106(6):1149–54.

    CAS  PubMed  Google Scholar 

  121. Zakowski M, Kaufman B, Berguson P, Tissot M, Yarmush L, Turndorf H. Esmolol use during resection of pheochromocytoma: report of three cases. Anesthesiology. 1989;70(5):875–7.

    Article  CAS  PubMed  Google Scholar 

  122. Chung PC, Ng YT, Hsieh JR, Yang MW, Li AH. Labetalol pretreatment reduces blood pressure instability during surgical resection of pheochromocytoma. J Formos Med Assoc. 2006;105(3):189–93.

    Article  CAS  PubMed  Google Scholar 

  123. Chung PC, Sum DC. Preliminary experience of using fixed dose of intravenous labetalol in surgical resection of pheochromocytoma. Ma Zui Xue Za Zhi. 1993;31(4):211–6.

    CAS  PubMed  Google Scholar 

  124. Roth JV. Use of vasopressin bolus and infusion to treat catecholamine-resistant hypotension during pheochromocytoma resection. Anesthesiology. 2007;106(4):883–4.

    Article  PubMed  Google Scholar 

  125. Amin Nasr A, Fatani J, Kashkari I, Al Shammary M, Amin T. Use of methylene blue in pheochromocytoma resection: case report. Paediatr Anaesth. 2009;19(4):396–401.

    Article  PubMed  Google Scholar 

  126. Amar L, Servais A, Gimenez-Roqueplo AP, Zinzindohoue F, Chatellier G, Plouin PF. Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma. J Clin Endocrinol Metab. 2005;90(4):2110–6.

    Article  CAS  PubMed  Google Scholar 

  127. Plouin PF, Amar L, Dekkers OM, Fassnacht M, Gimenez-Roqueplo AP, Lenders JW, et al. 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. 2016;174(5):G1–10.

    Article  CAS  PubMed  Google Scholar 

  128. Plouin PF, Chatellier G, Fofol I, Corvol P. Tumor recurrence and hypertension persistence after successful pheochromocytoma operation. Hypertension. 1997;29(5):1133–9.

    Article  CAS  PubMed  Google Scholar 

  129. Carrasquillo JA, Pandit-Taskar N, Chen CC. I-131 metaiodobenzylguanidine therapy of pheochromocytoma and paraganglioma. Semin Nucl Med. 2016;46(3):203–14.

    Article  PubMed  Google Scholar 

  130. van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP. (131)I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: systematic review and meta-analysis. Clin Endocrinol. 2014;80(4):487–501.

    Article  CAS  Google Scholar 

  131. Krempf M, Lumbroso J, Mornex R, Brendel AJ, Wemeau JL, Delisle MJ, et al. Use of m-[131I]iodobenzylguanidine in the treatment of malignant pheochromocytoma. J Clin Endocrinol Metab. 1991;72(2):455–61.

    Article  CAS  PubMed  Google Scholar 

  132. Sisson JC, Shapiro B, Beierwaltes WH, Glowniak JV, Nakajo M, Mangner TJ, et al. Radiopharmaceutical treatment of malignant pheochromocytoma. J Nucl Med. 1984;25(2):197–206.

    CAS  PubMed  Google Scholar 

  133. Solanki KK, Bomanji J, Moyes J, Mather SJ, Trainer PJ, Britton KE. A pharmacological guide to medicines which interfere with the biodistribution of radiolabelled meta-iodobenzylguanidine (MIBG). Nucl Med Commun. 1992;13(7):513–21.

    Article  CAS  PubMed  Google Scholar 

  134. Khafagi FA, Shapiro B, Fig LM, Mallette S, Sisson JC. Labetalol reduces iodine-131 MIBG uptake by pheochromocytoma and normal tissues. J Nucl Med. 1989;30(4):481–9.

    CAS  PubMed  Google Scholar 

  135. Friedman NC, Hassan A, Grady E, Matsuoka DT, Jacobson AF. Efficacy of thyroid blockade on thyroid radioiodine uptake in 123I-mIBG imaging. J Nucl Med. 2014;55(2):211–5.

    Article  CAS  PubMed  Google Scholar 

  136. Giammarile F, Chiti A, Lassmann M, Brans B, Flux G. Eanm. EANM procedure guidelines for 131I-meta-iodobenzylguanidine (131I-mIBG) therapy. Eur J Nucl Med Mol Imaging. 2008;35(5):1039–47.

    Article  CAS  PubMed  Google Scholar 

  137. Gedik GK, Hoefnagel CA, Bais E, Olmos RA. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2008;35(4):725–33.

    Article  CAS  PubMed  Google Scholar 

  138. Hartley A, Spooner D, Brunt AM. Management of malignant phaeochromocytoma: a retrospective review of the use of MIBG and chemotherapy in the West Midlands. Clin Oncol (R Coll Radiol). 2001;13(5):361–6.

    CAS  Google Scholar 

  139. Gonias S, Goldsby R, Matthay KK, Hawkins R, Price D, Huberty J, et al. Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma. J Clin Oncol. 2009;27(25):4162–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  140. Castellani MR, Seghezzi S, Chiesa C, Aliberti GL, Maccauro M, Seregni E, et al. (131)I-MIBG treatment of pheochromocytoma: low versus intermediate activity regimens of therapy. Q J Nucl Med Mol Imaging. 2010;54(1):100–13.

    CAS  PubMed  Google Scholar 

  141. Makis W, McCann K, McEwan AJ, Sawyer MB. Combined treatment with 131I-MIBG and sunitinib induces remission in a patient with metastatic paraganglioma due to hereditary paraganglioma-pheochromocytoma syndrome from an SDHB mutation. Clin Nucl Med. 2016;41(3):204–6.

    Article  PubMed  Google Scholar 

  142. Ayala-Ramirez M, Chougnet CN, Habra MA, Palmer JL, Leboulleux S, Cabanillas ME, et al. Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas. J Clin Endocrinol Metab. 2012;97(11):4040–50.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  143. Hata J, Haga N, Ishibashi K, Takahashi N, Ogawa S, Kataoka M, et al. Sunitinib for refractory malignant pheochromocytoma: two case reports. Int Urol Nephrol. 2014;46(7):1309–12.

    Article  PubMed  Google Scholar 

  144. Joshua AM, Ezzat S, Asa SL, Evans A, Broom R, Freeman M, et al. Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma. J Clin Endocrinol Metab. 2009;94(1):5–9.

    Article  CAS  PubMed  Google Scholar 

  145. Mundschenk J, Unger N, Schulz S, Hollt V, Schulz S, Steinke R, et al. Somatostatin receptor subtypes in human pheochromocytoma: subcellular expression pattern and functional relevance for octreotide scintigraphy. J Clin Endocrinol Metab. 2003;88(11):5150–7.

    Article  CAS  PubMed  Google Scholar 

  146. Koriyama N, Kakei M, Yaekura K, Okui H, Yamashita T, Nishimura H, et al. Control of catecholamine release and blood pressure with octreotide in a patient with pheochromocytoma: a case report with in vitro studies. Horm Res. 2000;53(1):46–50.

    Article  CAS  PubMed  Google Scholar 

  147. Kopf D, Bockisch A, Steinert H, Hahn K, Beyer J, Neumann HP, et al. Octreotide scintigraphy and catecholamine response to an octreotide challenge in malignant phaeochromocytoma. Clin Endocrinol (Oxf). 1997;46(1):39–44.

    Article  CAS  Google Scholar 

  148. Plouin PF, Bertherat J, Chatellier G, Billaud E, Azizi M, Grouzmann E, et al. Short-term effects of octreotide on blood pressure and plasma catecholamines and neuropeptide Y levels in patients with phaeochromocytoma: a placebo-controlled trial. Clin Endocrinol (Oxf). 1995;42(3):289–94.

    Article  CAS  Google Scholar 

  149. Lamarre-Cliche M, Gimenez-Roqueplo AP, Billaud E, Baudin E, Luton JP, Plouin PF. Effects of slow-release octreotide on urinary metanephrine excretion and plasma chromogranin A and catecholamine levels in patients with malignant or recurrent phaeochromocytoma. Clin Endocrinol (Oxf). 2002;57(5):629–34.

    Article  CAS  Google Scholar 

  150. Menda Y, O'Dorisio MS, Kao S, Khanna G, Michael S, Connolly M, et al. Phase I trial of 90Y-DOTATOC therapy in children and young adults with refractory solid tumors that express somatostatin receptors. J Nucl Med. 2010;51(10):1524–31.

    Article  PubMed  PubMed Central  Google Scholar 

  151. Forrer F, Riedweg I, Maecke HR, Mueller-Brand J. Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma. Q J Nucl Med Mol Imaging. 2008;52(4):334–40.

    CAS  PubMed  Google Scholar 

  152. van Essen M, Krenning EP, Kooij PP, Bakker WH, Feelders RA, de Herder WW, et al. Effects of therapy with [177Lu-DOTA0, Tyr3]octreotate in patients with paraganglioma, meningioma, small cell lung carcinoma, and melanoma. J Nucl Med. 2006;47(10):1599–606.

    PubMed  Google Scholar 

  153. Zovato S, Kumanova A, Dematte S, Sansovini M, Bodei L, Di Sarra D, et al. Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE in individuals with neck or mediastinal paraganglioma (PGL). Horm Metab Res. 2012;44(5):411–4.

    Article  CAS  PubMed  Google Scholar 

  154. Cecchin D, Schiavi F, Fanti S, Favero M, Manara R, Fassina A, et al. Peptide receptor radionuclide therapy in a case of multiple spinal canal and cranial paragangliomas. J Clin Oncol. 2011;29(7):e171–4.

    Article  PubMed  Google Scholar 

  155. Mamlouk MD, vanSonnenberg E, Stringfellow G, Smith D, Wendt A. Radiofrequency ablation and biopsy of metastatic pheochromocytoma: emphasizing safety issues and dangers. J Vasc Interv Radiol. 2009;20(5):670–3.

    Article  PubMed  Google Scholar 

  156. McBride JF, Atwell TD, Charboneau WJ, Young Jr WF, Wass TC, Callstrom MR. Minimally invasive treatment of metastatic pheochromocytoma and paraganglioma: efficacy and safety of radiofrequency ablation and cryoablation therapy. J Vasc Interv Radiol. 2011;22(9):1263–70.

    Article  PubMed  Google Scholar 

  157. Pacak K, Fojo T, Goldstein DS, Eisenhofer G, Walther MM, Linehan WM, et al. Radiofrequency ablation: a novel approach for treatment of metastatic pheochromocytoma. J Natl Cancer Inst. 2001;93(8):648–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  158. Venkatesan AM, Locklin J, Lai EW, Adams KT, Fojo AT, Pacak K, et al. Radiofrequency ablation of metastatic pheochromocytoma. J Vasc Interv Radiol. 2009;20(11):1483–90.

    Article  PubMed  PubMed Central  Google Scholar 

  159. Vogel J, Atanacio AS, Prodanov T, Turkbey BI, Adams K, Martucci V, et al. External beam radiation therapy in treatment of malignant pheochromocytoma and paraganglioma. Front Oncol. 2014;4:166.

    Article  PubMed  PubMed Central  Google Scholar 

  160. Kasliwal MK, Sharma MS, Vaishya S, Sharma BS. Metachronous pheochromocytoma metastasis to the upper dorsal spine-6-year survival. Spine J. 2008;8(5):845–8.

    Article  PubMed  Google Scholar 

  161. Scalfani MT, Arnold PM, Anderson KK. Metastatic adrenal pheochromocytoma to the thoracic spine. Coluna/Columna. 2010;9:343–6.

    Article  Google Scholar 

  162. Fishbein L, Bonner L, Torigian DA, Nathanson KL, Cohen DL, Pryma D, et al. External beam radiation therapy (EBRT) for patients with malignant pheochromocytoma and non-head and -neck paraganglioma: combination with 131I-MIBG. Horm Metab Res. 2012;44(5):405–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  163. Jackson CG, McGrew BM, Forest JA, Netterville JL, Hampf CF, Glasscock 3rd ME. Lateral skull base surgery for glomus tumors: long-term control. Otol Neurotol. 2001;22(3):377–82.

    Article  CAS  PubMed  Google Scholar 

  164. Chino JP, Sampson JH, Tucci DL, Brizel DM, Kirkpatrick JP. Paraganglioma of the head and neck: long-term local control with radiotherapy. Am J Clin Oncol. 2009;32(3):304–7.

    Article  PubMed  Google Scholar 

  165. Guss ZD, Batra S, Limb CJ, Li G, Sughrue ME, Redmond K, et al. Radiosurgery of glomus jugulare tumors: a meta-analysis. Int J Radiat Oncol Biol Phys. 2011;81(4):e497–502.

    Article  PubMed  PubMed Central  Google Scholar 

  166. Schuster D, Sweeney AD, Stavas MJ, Tawfik KY, Attia A, Cmelak AJ, et al. Initial radiographic tumor control is similar following single or multi-fractionated stereotactic radiosurgery for jugular paragangliomas. Am J Otolaryngol. 2016;37(3):255–8.

    Article  PubMed  Google Scholar 

  167. Hafez RF, Morgan MS, Fahmy OM. An intermediate term benefits and complications of gamma knife surgery in management of glomus jugulare tumor. World J Surg Oncol. 2016;14(1):36.

    Article  PubMed  PubMed Central  Google Scholar 

  168. Poznanovic SA, Cass SP, Kavanagh BD. Short-term tumor control and acute toxicity after stereotactic radiosurgery for glomus jugulare tumors. Otolaryngol Head Neck Surg. 2006;134(3):437–42.

    Article  PubMed  Google Scholar 

  169. Wegner RE, Rodriguez KD, Heron DE, Hirsch BE, Ferris RL, Burton SA. Linac-based stereotactic body radiation therapy for treatment of glomus jugulare tumors. Radiother Oncol. 2010;97(3):395–8.

    Article  PubMed  Google Scholar 

  170. Keiser HR, Goldstein DS, Wade JL, Douglas FL, Averbuch SD. Treatment of malignant pheochromocytoma with combination chemotherapy. Hypertension. 1985;7(3 Pt 2):I18–24.

    Article  CAS  PubMed  Google Scholar 

  171. Averbuch SD, Steakley CS, Young RC, Gelmann EP, Goldstein DS, Stull R, et al. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med. 1988;109(4):267–73.

    Article  CAS  PubMed  Google Scholar 

  172. Tanabe A, Naruse M, Nomura K, Tsuiki M, Tsumagari A, Ichihara A. Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine in patients with malignant pheochromocytoma and paraganglioma. Horm Cancer. 2013;4(2):103–10.

    Article  CAS  PubMed  Google Scholar 

  173. Huang H, Abraham J, Hung E, Averbuch S, Merino M, Steinberg SM, et al. Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer. 2008;113(8):2020–8.

    Article  CAS  PubMed  Google Scholar 

  174. Nomura K, Kimura H, Shimizu S, Kodama H, Okamoto T, Obara T, et al. Survival of patients with metastatic malignant pheochromocytoma and efficacy of combined cyclophosphamide, vincristine, and dacarbazine chemotherapy. J Clin Endocrinol Metab. 2009;94(8):2850–6.

    Article  CAS  PubMed  Google Scholar 

  175. Ayala-Ramirez M, Feng L, Habra MA, Rich T, Dickson PV, Perrier N, et al. Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience. Cancer. 2012;118(11):2804–12.

    Article  CAS  PubMed  Google Scholar 

  176. He J, Makey D, Fojo T, Adams KT, Havekes B, Eisenhofer G, et al. Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma. Endocrine. 2009;36(2):189–93.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  177. Sisson JC, Shapiro B, Shulkin BL, Urba S, Zempel S, Spaulding S. Treatment of malignant pheochromocytomas with 131-I metaiodobenzylguanidine and chemotherapy. Am J Clin Oncol. 1999;22(4):364–70.

    Article  CAS  PubMed  Google Scholar 

  178. Patel SR, Winchester DJ, Benjamin RS. A 15-year experience with chemotherapy of patients with paraganglioma. Cancer. 1995;76(8):1476–80.

    Article  CAS  PubMed  Google Scholar 

  179. Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, et al. SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Int J Cancer. 2014;135(11):2711–20.

    Article  CAS  PubMed  Google Scholar 

Download references

Disclosure Statement

The authors have nothing to disclose.

Author information

Authors and Affiliations

  1. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, CRC, 1E-3140, 10 Center Drive, MSC-1109, Bethesda, MD, 20892-1109, USA

    Garima Gupta M.D. & Karel Pacak M.D., Ph.D., D.Sc., F.A.C.E.

  2. Division of Endocrinology, Department of Medicine, University of Connecticut Health Center, Farmington, CT, USA

    Vitaly Kantorovich M.D.

Authors
  1. Garima Gupta M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Vitaly Kantorovich M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Karel Pacak M.D., Ph.D., D.Sc., F.A.C.E.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Karel Pacak M.D., Ph.D., D.Sc., F.A.C.E. .

Editor information

Editors and Affiliations

  1. Endocrine Oncology Branch, National Institutes of Health National Cancer Institute, Bethesda, Maryland, USA

    Electron Kebebew

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Gupta, G., Kantorovich, V., Pacak, K. (2017). Management of Pheochromocytoma and Paraganglioma. In: Kebebew, E. (eds) Management of Adrenal Masses in Children and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-44136-8_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-44136-8_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-44134-4

  • Online ISBN: 978-3-319-44136-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation