Advertisement

Medical Management of Pheochromocytoma

  • Daniel J. Toft
  • Mark E. Molitch
Chapter
Part of the Contemporary Endocrinology book series (COE)

Abstract

Patients with catecholamine-secreting pheochromocytomas and paragangliomas require medical treatment to control blood pressure and tachyarrhythmias and to prepare the patient for surgical tumor resection. The mainstay of medical treatment is α-adrenergic receptor blockade, and both nonselective and α1-selective adrenergic receptor blockers can be used. Once blood pressure control is achieved, β-adrenergic receptor blockers can be added to control tachycardia and arrhythmias. When blood pressure cannot be achieved by α-blockade alone, calcium channel blockers and metyrosine can be added. Such treatment, along with fluid and salt replacement, should be carried out for 7–14 days prior to surgery to avoid postoperative hypotension. Uncommonly, patients may present with hypertensive emergencies and require ICU admission with intravenous α-adrenergic receptor blockade with phentolamine and other antihypertensive agents. Another uncommon presentation is acute heart failure which can rapidly progress to cardiogenic shock; such patients also require ICU admission and rapid institution of α- and then β-adrenergic receptor blockade. Patients with metastatic disease may require chronic adrenergic receptor blockade, and new chemotherapeutic agents have varying success. Overall, medical therapy with subsequent surgery of patients with pheochromocytomas is quite successful, and most patients do very well.

Keywords

Pheochromocytoma Paraganglioma Adrenergic Phenoxybenzamine Hypertension 

References

  1. 1.
    Challis BG, Casey RT, Simpson HL, Gurnell M. Is there an optimal preoperative management strategy for phaeochromocytoma/paraganglioma? Clin Endocrinol. 2017;86(2):163–7.  https://doi.org/10.1111/cen.13252.CrossRefGoogle Scholar
  2. 2.
    Gunawardane PT, Grossman A. Phaeochromocytoma and Paraganglioma. Adv Exp Med Biol. 2016.  https://doi.org/10.1007/5584_2016_76.
  3. 3.
    Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr, Endocrine S. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915–42.  https://doi.org/10.1210/jc.2014-1498.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Lentschener C, Gaujoux S, Tesniere A, Dousset B. Point of controversy: perioperative care of patients undergoing pheochromocytoma removal-time for a reappraisal? Eur J Endocrinol. 2011;165(3):365–73.  https://doi.org/10.1530/EJE-11-0162.CrossRefPubMedGoogle Scholar
  5. 5.
    Livingstone M, Duttchen K, Thompson J, Sunderani Z, Hawboldt G, Sarah Rose M, Pasieka J. Hemodynamic Stability During Pheochromocytoma Resection: Lessons Learned Over the Last Two Decades. Ann Surg Oncol. 2015;22(13):4175–80.  https://doi.org/10.1245/s10434-015-4519-y.CrossRefPubMedGoogle Scholar
  6. 6.
    Chang RY, Lang BH, Wong KP, Lo CY. High pre-operative urinary norepinephrine is an independent determinant of peri-operative hemodynamic instability in unilateral pheochromocytoma/paraganglioma removal. World J Surg. 2014;38(9):2317–23.  https://doi.org/10.1007/s00268-014-2597-9.CrossRefPubMedGoogle Scholar
  7. 7.
    Weingarten TN, Welch TL, Moore TL, Walters GF, Whipple JL, Cavalcante A, Bancos I, Young WF Jr, Gruber LM, Shah MZ, McKenzie TJ, Schroeder DR, Sprung J. Preoperative Levels of Catecholamines and Metanephrines and Intraoperative Hemodynamics of Patients Undergoing Pheochromocytoma and Paraganglioma Resection. Urology. 2017;100:131–8.  https://doi.org/10.1016/j.urology.2016.10.012.CrossRefPubMedGoogle Scholar
  8. 8.
    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.  https://doi.org/10.1007/s00268-002-6667-z.CrossRefPubMedGoogle Scholar
  9. 9.
    Li J, Yang CH. Improvement of preoperative management in patients with adrenal pheochromocytoma. Int J Clin Exp Med. 2014;7(12):5541–6.PubMedPubMedCentralGoogle Scholar
  10. 10.
    Zhu Y, He HC, Su TW, Wu YX, Wang WQ, Zhao JP, Shen Z, Zhang CY, Rui WB, Zhou WL, Sun FK, Ning G. Selective alpha1-adrenoceptor antagonist (controlled release tablets) in preoperative management of pheochromocytoma. Endocrine. 2010;38(2):254–9.  https://doi.org/10.1007/s12020-010-9381-x.CrossRefPubMedGoogle Scholar
  11. 11.
    Agrawal R, Mishra SK, Bhatia E, Mishra A, Chand G, Agarwal G, Agarwal A, Verma AK. Prospective study to compare peri-operative hemodynamic alterations following preparation for pheochromocytoma surgery by phenoxybenzamine or prazosin. World J Surg. 2014;38(3):716–23.  https://doi.org/10.1007/s00268-013-2325-x.CrossRefPubMedGoogle Scholar
  12. 12.
    Kocak S, Aydintug S, Canakci N. Alpha blockade in preoperative preparation of patients with pheochromocytomas. Int Surg. 2002;87(3):191–4.PubMedGoogle Scholar
  13. 13.
    Kiernan CM, Du L, Chen X, Broome JT, Shi C, Peters MF, Solorzano CC. Predictors of hemodynamic instability during surgery for pheochromocytoma. Ann Surg Oncol. 2014;21(12):3865–71.  https://doi.org/10.1245/s10434-014-3847-7.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Steinsapir J, Carr AA, Prisant LM, Bransome ED Jr. Metyrosine and pheochromocytoma. Arch Intern Med. 1997;157(8):901–6.CrossRefGoogle Scholar
  15. 15.
    Wachtel H, Kennedy EH, Zaheer S, Bartlett EK, Fishbein L, Roses RE, Fraker DL, Cohen DL. Preoperative Metyrosine Improves Cardiovascular Outcomes for Patients Undergoing Surgery for Pheochromocytoma and Paraganglioma. Ann Surg Oncol. 2015;22(Suppl 3):S646–54.  https://doi.org/10.1245/s10434-015-4862-z.CrossRefPubMedGoogle Scholar
  16. 16.
    Fishbein L, Orlowski R, Cohen D. Pheochromocytoma/Paraganglioma: Review of perioperative management of blood pressure and update on genetic mutations associated with pheochromocytoma. J Clin Hypertens (Greenwich). 2013;15(6):428–34.  https://doi.org/10.1111/jch.12084.CrossRefGoogle Scholar
  17. 17.
    Brunaud L, Boutami M, Nguyen-Thi PL, Finnerty B, Germain A, Weryha G, Fahey TJ 3rd, Mirallie E, Bresler L, Zarnegar R. Both preoperative alpha and calcium channel blockade impact intraoperative hemodynamic stability similarly in the management of pheochromocytoma. Surgery. 2014;156(6):1410–7. discussion1417-1418.  https://doi.org/10.1016/j.surg.2014.08.022.CrossRefPubMedGoogle Scholar
  18. 18.
    Mazza A, Armigliato M, Marzola MC, Schiavon L, Montemurro D, Vescovo G, Zuin M, Chondrogiannis S, Ravenni R, Opocher G, Colletti PM, Rubello D. Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features. Endocrine. 2014;45(3):469–78.  https://doi.org/10.1007/s12020-013-0007-y.CrossRefPubMedGoogle Scholar
  19. 19.
    Cubeddu LX, Fuenmayor N, Varin F, Villagra VG, Colindres RE, Powell JR. Mechanism of the vasodilatory effect of carvedilol in normal volunteers: a comparison with labetalol. J Cardiovasc Pharmacol. 1987;10(Suppl 11):S81–4.PubMedGoogle Scholar
  20. 20.
    Giannattasio C, Cattaneo BM, Seravalle G, Carugo S, Mangoni AA, Grassi G, Zanchetti A, Mancia G. Alpha 1-blocking properties of carvedilol during acute and chronic administration. J Cardiovasc Pharmacol. 1992;19(Suppl 1):S18–22.CrossRefGoogle Scholar
  21. 21.
    Shao Y, Chen R, Shen ZJ, Teng Y, Huang P, Rui WB, Xie X, Zhou WL. Preoperative alpha blockade for normotensive pheochromocytoma: is it necessary? J Hypertens. 2011;29(12):2429–32.  https://doi.org/10.1097/HJH.0b013e32834d24d9.CrossRefPubMedGoogle Scholar
  22. 22.
    Lafont M, Fagour C, Haissaguerre M, Darancette G, Wagner T, Corcuff JB, Tabarin A. Per-operative hemodynamic instability in normotensive patients with incidentally discovered pheochromocytomas. J Clin Endocrinol Metab. 2015;100(2):417–21.  https://doi.org/10.1210/jc.2014-2998.CrossRefPubMedGoogle Scholar
  23. 23.
    Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens. 2011;29(11):2049–60.  https://doi.org/10.1097/HJH.0b013e32834a4ce9.CrossRefPubMedGoogle Scholar
  24. 24.
    Giavarini A, Chedid A, Bobrie G, Plouin PF, Hagege A, Amar L. Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma. Heart. 2013;99(19):1438–44.  https://doi.org/10.1136/heartjnl-2013-304073.CrossRefPubMedGoogle Scholar
  25. 25.
    Batisse-Lignier M, Pereira B, Motreff P, Pierrard R, Burnot C, Vorilhon C, Maqdasy S, Roche B, Desbiez F, Clerfond G, Citron B, Lusson JR, Tauveron I, Eschalier R. Acute and Chronic Pheochromocytoma-Induced Cardiomyopathies: Different Prognoses?: A Systematic Analytical Review. Medicine (Baltimore). 2015;94(50):e2198.  https://doi.org/10.1097/MD.0000000000002198.CrossRefGoogle Scholar
  26. 26.
    Kassim TA, Clarke DD, Mai VQ, Clyde PW, Mohamed Shakir KM. Catecholamine-induced cardiomyopathy. Endocr Pract. 2008;14(9):1137–49.  https://doi.org/10.4158/EP.14.9.1137.CrossRefPubMedGoogle Scholar
  27. 27.
    Poirier E, Thauvette D, Hogue JC. Management of exclusively dopamine-secreting abdominal pheochromocytomas. J Am Coll Surg. 2013;216(2):340–6.  https://doi.org/10.1016/j.jamcollsurg.2012.10.002.CrossRefPubMedGoogle Scholar
  28. 28.
    Conzo G, Musella M, Corcione F, De Palma M, Ferraro F, Palazzo A, Napolitano S, Milone M, Pasquali D, Sinisi AA, Colantuoni V, Santini L. Laparoscopic adrenalectomy, a safe procedure for pheochromocytoma. A retrospective review of clinical series. Int J Surg. 2013;11(2):152–6.  https://doi.org/10.1016/j.ijsu.2012.12.007.CrossRefPubMedGoogle Scholar
  29. 29.
    Riester A, Weismann D, Quinkler M, Lichtenauer UD, Sommerey S, Halbritter R, Penning R, Spitzweg C, Schopohl J, Beuschlein F, Reincke M. Life-threatening events in patients with pheochromocytoma. Eur J Endocrinol. 2015;173(6):757–64.  https://doi.org/10.1530/EJE-15-0483.CrossRefPubMedGoogle Scholar
  30. 30.
    Mohamed HA, Aldakar MO, Habib N. Cardiogenic shock due to acute hemorrhagic necrosis of a pheochromocytoma: a case report and review of the literature. Can J Cardiol. 2003;19(5):573–6.PubMedGoogle Scholar
  31. 31.
    Hosseinnezhad A, Black RM, Aeddula NR, Adhikari D, Trivedi N. Glucagon-induced pheochromocytoma crisis. Endocr Pract. 2011;17(3):e51–4.  https://doi.org/10.4158/EP10388.CR.CrossRefPubMedGoogle Scholar
  32. 32.
    Preuss J, Woenckhaus C, Schwesinger G, Madea B. Non-diagnosed pheochromocytoma as a cause of sudden death in a 49-year-old man: a case report with medico-legal implications. Forensic Sci Int. 2006;156(2–3):223–8.  https://doi.org/10.1016/j.forsciint.2005.05.025.CrossRefPubMedGoogle Scholar
  33. 33.
    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.  https://doi.org/10.1530/EJE-07-0778.CrossRefPubMedGoogle Scholar
  34. 34.
    Hemmady P, Snyder RW, Klink M. A hypertensive emergency following cataract extraction and cornea transplantation. Ophthalmic Surg. 1989;20(9):647–9. Discussion, 650PubMedGoogle Scholar
  35. 35.
    Pineda Pompa LR, Barrera-Ramirez CF, Martinez-Valdez J, Rodriguez PD, Guzman CE. Pheochromocytoma-induced acute pulmonary edema and reversible catecholamine cardiomyopathy mimicking acute myocardial infarction. Rev Port Cardiol. 2004;23(4):561–8.PubMedGoogle Scholar
  36. 36.
    Casola G, Nicolet V, vanSonnenberg E, Withers C, Bretagnolle M, Saba RM, Bret PM. Unsuspected pheochromocytoma: risk of blood-pressure alterations during percutaneous adrenal biopsy. Radiology. 1986;159(3):733–5.  https://doi.org/10.1148/radiology.159.3.3517958.CrossRefPubMedGoogle Scholar
  37. 37.
    Iio K, Sakurai S, Kato T, Nishiyama S, Hata T, Mawatari E, Suzuki C, Takekoshi K, Higuchi K, Aizawa T, Ikeda U. Endomyocardial biopsy in a patient with hemorrhagic pheochromocytoma presenting as inverted Takotsubo cardiomyopathy. Heart Vessel. 2013;28(2):255–63.  https://doi.org/10.1007/s00380-012-0247-4.CrossRefGoogle Scholar
  38. 38.
    Kim S, Yu A, Filippone LA, Kolansky DM, Raina A. Inverted-Takotsubo pattern cardiomyopathy secondary to pheochromocytoma: a clinical case and literature review. Clin Cardiol. 2010;33(4):200–5.  https://doi.org/10.1002/clc.20680.CrossRefPubMedGoogle Scholar
  39. 39.
    Sanchez-Recalde A, Costero O, Oliver JM, Iborra C, Ruiz E, Sobrino JA. Images in cardiovascular medicine. Pheochromocytoma-related cardiomyopathy: inverted Takotsubo contractile pattern. Circulation. 2006;113(17):e738–9.  https://doi.org/10.1161/CIRCULATIONAHA.105.581108.CrossRefPubMedGoogle Scholar
  40. 40.
    Subramanyam S, Kreisberg RA. Pheochromocytoma: a cause of ST-segment elevation myocardial infarction, transient left ventricular dysfunction, and takotsubo cardiomyopathy. Endocr Pract. 2012;18(4):e77–80.  https://doi.org/10.4158/EP11346.CR.CrossRefPubMedGoogle Scholar
  41. 41.
    Brouwers FM, Eisenhofer G, Lenders JW, Pacak K. Emergencies caused by pheochromocytoma, neuroblastoma, or ganglioneuroma. Endocrinol Metab Clin N Am. 2006;35(4):699–724., viii.  https://doi.org/10.1016/j.ecl.2006.09.014.CrossRefGoogle Scholar
  42. 42.
    Whitelaw BC, Prague JK, Mustafa OG, Schulte KM, Hopkins PA, Gilbert JA, McGregor AM, Aylwin SJ. Phaeochromocytoma [corrected] crisis. Clin Endocrinol. 2014;80(1):13–22.  https://doi.org/10.1111/cen.12324.CrossRefGoogle Scholar
  43. 43.
    Gabrielson GV, Guffin AV, Kaplan JA, Pertsemlidis D, Iberti TJ. Continuous intravenous infusions of phentolamine and esmolol for preoperative and intraoperative adrenergic blockade in patients with pheochromocytoma. J Cardiothorac Anesth. 1987;1(6):554–8.CrossRefGoogle Scholar
  44. 44.
    McMillian WD, Trombley BJ, Charash WE, Christian RC. Phentolamine continuous infusion in a patient with pheochromocytoma. Am J Health Syst Pharm. 2011;68(2):130–4.  https://doi.org/10.2146/ajhp090619.CrossRefPubMedGoogle Scholar
  45. 45.
    Habbe N, Ruger F, Bojunga J, Bechstein WO, Holzer K. Urapidil in the preoperative treatment of pheochromocytomas: a safe and cost-effective method. World J Surg. 2013;37(5):1141–6.  https://doi.org/10.1007/s00268-013-1933-9.CrossRefPubMedGoogle Scholar
  46. 46.
    Miura Y, Yoshinaga K, Fukuchi S, Kikawada R, Kuramoto K, Takeuchi T, Satoh T. Antihypertensive efficacy and safety of urapidil, alone or in combination with beta-blockers, in patients with phaeochromocytoma. J Hypertens Suppl. 1988;6(2):S59–62.PubMedGoogle Scholar
  47. 47.
    Arai T, Hatano Y, Ishida H, Mori K. Use of nicardipine in the anesthetic management of pheochromocytoma. Anesth Analg. 1986;65(6):706–8.CrossRefGoogle Scholar
  48. 48.
    Proye C, Thevenin D, Cecat P, Petillot P, Carnaille B, Verin P, Sautier M, Racadot N. 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.PubMedGoogle Scholar
  49. 49.
    James MF, Cronje L. Pheochromocytoma crisis: the use of magnesium sulfate. Anesth Analg. 2004;99(3):680–6., table of contents.  https://doi.org/10.1213/01.ane.0000133136.01381.52.CrossRefPubMedPubMedCentralGoogle Scholar
  50. 50.
    Scholten A, Cisco RM, Vriens MR, Cohen JK, Mitmaker EJ, Liu C, Tyrrell JB, Shen WT, Duh QY. Pheochromocytoma crisis is not a surgical emergency. J Clin Endocrinol Metab. 2013;98(2):581–91.  https://doi.org/10.1210/jc.2012-3020.CrossRefPubMedGoogle Scholar
  51. 51.
    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.CrossRefGoogle Scholar
  52. 52.
    Niemeijer ND, Alblas G, van Hulsteijn LT, Dekkers OM, Corssmit EP. Chemotherapy with cyclophosphamide, vincristine and dacarbazine for malignant paraganglioma and pheochromocytoma: systematic review and meta-analysis. Clin Endocrinol. 2014;81(5):642–51.  https://doi.org/10.1111/cen.12542.CrossRefGoogle Scholar
  53. 53.
    Huang H, Abraham J, Hung E, Averbuch S, Merino M, Steinberg SM, Pacak K, Fojo T. 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.  https://doi.org/10.1002/cncr.23812.CrossRefPubMedGoogle Scholar
  54. 54.
    Asai S, Katabami T, Tsuiki M, Tanaka Y, Naruse M. Controlling tumor progression with cyclophosphamide, vincristine, and dacarbazine treatment improves survival in patients with metastatic and unresectable malignant pheochromocytomas/paragangliomas. Horm Cancer. 2017.  https://doi.org/10.1007/s12672-017-0284-7.
  55. 55.
    Nomura K, Kimura H, Shimizu S, Kodama H, Okamoto T, Obara T, Takano K. 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.  https://doi.org/10.1210/jc.2008-2697.CrossRefPubMedGoogle Scholar
  56. 56.
    Kulke MH, Stuart K, Enzinger PC, Ryan DP, Clark JW, Muzikansky A, Vincitore M, Michelini A, Fuchs CS. Phase II study of temozolomide and thalidomide in patients with metastatic neuroendocrine tumors. J Clin Oncol. 2006;24(3):401–6.  https://doi.org/10.1200/JCO.2005.03.6046.CrossRefPubMedGoogle Scholar
  57. 57.
    Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, Deandreis D, Borget I, Loriot C, Chougnet C, Letouze E, Young J, Amar L, Bertherat J, Libe R, Dumont F, Deschamps F, Schlumberger M, Gimenez-Roqueplo AP, Baudin E. SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Int J Cancer. 2014;135(11):2711–20.  https://doi.org/10.1002/ijc.28913.CrossRefPubMedGoogle Scholar
  58. 58.
    Nakajo M, Shapiro B, Copp J, Kalff V, Gross MD, Sisson JC, Beierwaltes WH. The normal and abnormal distribution of the adrenomedullary imaging agent m-[I-131]iodobenzylguanidine (I-131 MIBG) in man: evaluation by scintigraphy. J Nucl Med. 1983;24(8):672–82.PubMedGoogle Scholar
  59. 59.
    Kaltsas GA, Mukherjee JJ, Foley R, Britton KE, Grossman AB. Treatment of Metastatic Pheochromocytoma and Paraganglioma With 131I-Meta-Iodobenzylguanidine (MIBG). Endocrinologist. 2003;13(4):321–33.CrossRefGoogle Scholar
  60. 60.
    Gonias S, Goldsby R, Matthay KK, Hawkins R, Price D, Huberty J, Damon L, Linker C, Sznewajs A, Shiboski S, Fitzgerald P. Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma. J Clin Oncol. 2009;27(25):4162–8.  https://doi.org/10.1200/JCO.2008.21.3496.CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Krempf M, Lumbroso J, Mornex R, Brendel AJ, Wemeau JL, Delisle MJ, Aubert B, Carpentier P, Fleury-Goyon MC, Gibold C, et al. Treatment of malignant pheochromocytoma with [131I]metaiodobenzylguanidine: a French multicenter study. J Nucl Biol Med (Turin Italy: 1991). 1991;35(4):284–7.Google Scholar
  62. 62.
    Loh KC, Fitzgerald PA, Matthay KK, Yeo PP, Price DC. The treatment of malignant pheochromocytoma with iodine-131 metaiodobenzylguanidine (131I-MIBG): a comprehensive review of 116 reported patients. J Endocrinol Investig. 1997;20(11):648–58.CrossRefGoogle Scholar
  63. 63.
    Troncone L, Rufini V, Daidone MS, De Santis M, Luzi S. [131I]metaiodobenzylguanidine treatment of malignant pheochromocytoma: experience of the Rome group. J Nucl Biol Med (Turin Italy: 1991). 1991;35(4):295–9.Google Scholar
  64. 64.
    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.  https://doi.org/10.1111/cen.12341.CrossRefGoogle Scholar
  65. 65.
    Fitzgerald PA, Goldsby RE, Huberty JP, Price DC, Hawkins RA, Veatch JJ, Dela Cruz F, Jahan TM, Linker CA, Damon L, Matthay KK. Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG). Ann N Y Acad Sci. 2006;1073:465–90.  https://doi.org/10.1196/annals.1353.050.CrossRefPubMedGoogle Scholar
  66. 66.
    Sze WC, Grossman AB, Goddard I, Amendra D, Shieh SC, Plowman PN, Drake WM, Akker SA, Druce MR. Sequelae and survivorship in patients treated with (131)I-MIBG therapy. Br J Cancer. 2013;109(3):565–72.  https://doi.org/10.1038/bjc.2013.365.CrossRefPubMedPubMedCentralGoogle Scholar
  67. 67.
    Rutherford MA, Rankin AJ, Yates TM, Mark PB, Perry CG, Reed NS, Freel EM. Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre. QJM. 2015;108(5):361–8.  https://doi.org/10.1093/qjmed/hcu208.CrossRefPubMedGoogle Scholar
  68. 68.
    Safford SD, Coleman RE, Gockerman JP, Moore J, Feldman JM, Leight GS Jr, Tyler DS, Olson JA Jr. Iodine −131 metaiodobenzylguanidine is an effective treatment for malignant pheochromocytoma and paraganglioma. Surgery. 2003;134(6):956–62.; discussion 962-953.  https://doi.org/10.1016/S0039.CrossRefPubMedGoogle Scholar
  69. 69.
    Reubi JC, Waser B, Khosla S, Kvols L, Goellner JR, Krenning E, Lamberts S. In vitro and in vivo detection of somatostatin receptors in pheochromocytomas and paragangliomas. J Clin Endocrinol Metab. 1992;74(5):1082–9.  https://doi.org/10.1210/jcem.74.5.1349024.CrossRefPubMedGoogle Scholar
  70. 70.
    Plouin PF, Bertherat J, Chatellier G, Billaud E, Azizi M, Grouzmann E, Epelbaum J. 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. 1995;42(3):289–94.CrossRefGoogle Scholar
  71. 71.
    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.PubMedGoogle Scholar
  72. 72.
    Medina-Ornelas SS, Garcia-Perez FO. Effectiveness of radiolabelled somatostatin analogues (90Y-DOTATOC and 177Lu-DOTATATE) in patients with metastatic neuroendocrine tumours: a single centre experience in Mexico. Rev Esp Med Nucl Imagen Mol. 2016.  https://doi.org/10.1016/j.remn.2016.09.005.
  73. 73.
    Zovato S, Kumanova A, Dematte S, Sansovini M, Bodei L, Di Sarra D, Casagranda E, Severi S, Ambrosetti A, Schiavi F, Opocher G, Paganelli G. Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE in individuals with neck or mediastinal paraganglioma (PGL). Horm Metab Res. 2012;44(5):411–4.  https://doi.org/10.1055/s-0032-1311637.CrossRefPubMedGoogle Scholar
  74. 74.
    Puranik AD, Kulkarni HR, Singh A, Baum RP. Peptide receptor radionuclide therapy with (90)Y/ (177)Lu-labelled peptides for inoperable head and neck paragangliomas (glomus tumours). Eur J Nucl Med Mol Imaging. 2015;42(8):1223–30.  https://doi.org/10.1007/s00259-015-3029-2.CrossRefPubMedGoogle Scholar
  75. 75.
    Gulenchyn KY, Yao X, Asa SL, Singh S, Law C. Radionuclide therapy in neuroendocrine tumours: a systematic review. Clin Oncol (R Coll Radiol). 2012;24(4):294–308.  https://doi.org/10.1016/j.clon.2011.12.003.CrossRefGoogle Scholar
  76. 76.
    Castinetti F, Kroiss A, Kumar R, Pacak K, Taieb D. 15 YEARS OF PARAGANGLIOMA: Imaging and imaging-based treatment of pheochromocytoma and paraganglioma. Endocr Relat Cancer. 2015;22(4):T135–45.  https://doi.org/10.1530/ERC-15-0175.CrossRefPubMedGoogle Scholar
  77. 77.
    Druce MR, Kaltsas GA, Fraenkel M, Gross DJ, Grossman AB. Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001). Horm Metab Res. 2009;41(9):697–702.  https://doi.org/10.1055/s-0029-1220687.CrossRefPubMedGoogle Scholar
  78. 78.
    Oh DY, Kim TW, Park YS, Shin SJ, Shin SH, Song EK, Lee HJ, Lee KW, Bang YJ. Phase 2 study of everolimus monotherapy in patients with nonfunctioning neuroendocrine tumors or pheochromocytomas/paragangliomas. Cancer. 2012;118(24):6162–70.  https://doi.org/10.1002/cncr.27675.CrossRefPubMedGoogle Scholar
  79. 79.
    McBride JF, Atwell TD, Charboneau WJ, Young WF Jr, 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.  https://doi.org/10.1016/j.jvir.2011.06.016.CrossRefPubMedGoogle Scholar
  80. 80.
    Venkatesan AM, Locklin J, Lai EW, Adams KT, Fojo AT, Pacak K, Wood BJ. Radiofrequency ablation of metastatic pheochromocytoma. J Vasc Interv Radiol. 2009;20(11):1483–90.  https://doi.org/10.1016/j.jvir.2009.07.031.CrossRefPubMedPubMedCentralGoogle Scholar
  81. 81.
    Pacak K, Fojo T, Goldstein DS, Eisenhofer G, Walther MM, Linehan WM, Bachenheimer L, Abraham J, Wood BJ. Radiofrequency ablation: a novel approach for treatment of metastatic pheochromocytoma. J Natl Cancer Inst. 2001;93(8):648–9.CrossRefGoogle Scholar
  82. 82.
    Ayala-Ramirez M, Chougnet CN, Habra MA, Palmer JL, Leboulleux S, Cabanillas ME, Caramella C, Anderson P, Al Ghuzlan A, Waguespack SG, Deandreis D, Baudin E, Jimenez C. Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas. J Clin Endocrinol Metab. 2012;97(11):4040–50.  https://doi.org/10.1210/jc.2012-2356.CrossRefPubMedPubMedCentralGoogle Scholar
  83. 83.
    Madhukar NS, Elemento O, Benes CH, Garnett MJ, Stein M, Bertino JR, Kaufman HL, Arrillaga-Romany I, Batchelor TT, Schalop L, Oster W, Stogniew M, Andreeff M, El-Deiry WS, Allen JE. Abstract LB-209: D2-like dopamine receptor antagonism by ONC201 identified by confluence of computational, receptor binding, and clinical studies. Cancer Res. 2016;76(14 Supplement):LB-209-LB-209.  https://doi.org/10.1158/1538-7445.am2016-lb-209.CrossRefGoogle Scholar
  84. 84.
    Allen JE, Krigsfeld G, Mayes PA, Patel L, Dicker DT, Patel AS, Dolloff NG, Messaris E, Scata KA, Wang W, Zhou JY, Wu GS, El-Deiry WS. Dual inactivation of Akt and ERK by TIC10 signals Foxo3a nuclear translocation, TRAIL gene induction, and potent antitumor effects. Sci Transl Med. 2013;5(171):171ra117.  https://doi.org/10.1126/scitranslmed.3004828.CrossRefGoogle Scholar
  85. 85.
    Saveanu A, Sebag F, Guillet B, Archange C, Essamet W, Barlier A, Palazzo FF, Taieb D. Targeting dopamine receptors subtype 2 (D2DR) in pheochromocytomas: head-to-head comparison between in vitro and in vivo findings. J Clin Endocrinol Metab. 2013;98(12):E1951–5.  https://doi.org/10.1210/jc.2013-2269.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Endocrinology, Metabolism and Molecular MedicineNorthwestern University Feinberg School of MedicineChicagoUSA

Personalised recommendations