Phaeochromocytoma

Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65. Abstract

Fishbein L, Del Rivero J, Else T, et al. The North American Neuroendocrine Tumor Society consensus guidelines for surveillance and management of metastatic and/or unresectable pheochromocytoma and paraganglioma. Pancreas. 2021 Apr 1;50(4):469-93.Full text  Abstract

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Full text  Abstract

Plouin PF, Amar L, Dekkers OM, et al; Guideline Working Group. 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 May;174(5):G1-10.Full text  Abstract

1. Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65. Abstract

2. Tevosian SG, Ghayee HK. Pheochromocytomas and paragangliomas. Endocrinol Metab Clin North Am. 2019 Dec;48(4):727-50. Abstract

3. Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014 Jan-Feb;38(1):7-41.Full text  Abstract

4. Fishbein L, Merrill S, Fraker DL, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. 2013 May;20(5):1444-50.Full text  Abstract

5. Liu P, Li M, Guan X, et al. Clinical syndromes and genetic screening strategies of pheochromocytoma and paraganglioma. J Kidney Cancer VHL. 2018 Dec 27;5(4):14-22. Abstract

6. Lloyd RV, Osamura RY, Klöppel G, et al, eds. WHO classification of tumours of endocrine organs: WHO classification of tumours. 4th ed., Vol. 10. Lyon, France: International Agency of research on Cancer (IARC); 2017.

7. Lam AK. Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine tumours. Endocr Pathol. 2017 Sep;28(3):213-27. Abstract

8. Gabi JN, Milhem MM, Tovar YE, et al. Severe Cushing syndrome due to an ACTH-producing pheochromocytoma: a case presentation and review of the literature. J Endocr Soc. 2018 May 24;2(7):621-30.Full text  Abstract

9. Jiang J, Zhang L, Wu Z, et al. A rare case of watery diarrhea, hypokalemia and achlorhydria syndrome caused by pheochromocytoma. BMC Cancer. 2014 Jul 31;14:553.Full text  Abstract

10. Ronen JA, Gavin M, Ruppert MD, et al. Glycemic disturbances in pheochromocytoma and paraganglioma. Cureus. 2019 Apr 27;11(4):e4551.Full text  Abstract

11. Sinnott B, Wu S, Sarne D, et al. Presentation of an unsuspected pheochromocytoma as acute aortic valvular insufficiency and diabetes mellitus type 2. Endocrinologist. 2006 Nov/Dec;16(6):335-9.

12. Davison AS, Jones DM, Ruthven S, et al. Clinical evaluation and treatment of phaeochromocytoma. Ann Clin Biochem. 2018 Jan;55(1):34-48.Full text  Abstract

13. Ebbehoj A, Stochholm K, Jacobsen SF, et al. Incidence and clinical presentation of pheochromocytoma and sympathetic paraganglioma: a population-based study. J Clin Endocrinol Metab. 2021 Apr 23;106(5):e2251-61.Full text  Abstract

14. Berends AMA, Buitenwerf E, de Krijger RR, et al. Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: a nationwide study and systematic review. Eur J Intern Med. 2018 May;51:68-73. Abstract

15. Plouin PF, Gimenez-Roqueplo AP. Pheochromocytomas and secreting paragangliomas. Orphanet J Rare Dis. 2006 Dec 8;1:49.Full text  Abstract

16. Leung AA, Pasieka JL, Hyrcza MD, et al. Epidemiology of pheochromocytoma and paraganglioma: population-based cohort study. Eur J Endocrinol. 2021 Jan;184(1):19-28.Full text  Abstract

17. Kim JH, Moon H, Noh J, et al. Epidemiology and prognosis of pheochromocytoma/paraganglioma in Korea: A nationwide study based on the National Health Insurance Service. Endocrinol Metab (Seoul). 2020 Mar;35(1):157-64.Full text  Abstract

18. Chen H, Sippel RS, O'Dorisio MS, 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 Aug;39(6):775-83.Full text  Abstract

19. Ebbehoj A, Li D, Kaur RJ, et al. Epidemiology of adrenal tumours in Olmsted County, Minnesota, USA: a population-based cohort study. Lancet Diabetes Endocrinol. 2020 Nov;8(11):894-902.Full text  Abstract

20. Moon JK, Mattei P. Pheochromocytoma and paraganglioma. Semin Pediatr Surg. 2020 Jun;29(3):150926. Abstract

21. Jhawar S, Arakawa Y, Kumar S, et al. New insights on the genetics of pheochromocytoma and paraganglioma and its clinical implications. Cancers (Basel). 2022 Jan 25;14(3):594.Full text  Abstract

22. Dariane C, Goncalves J, Timsit MO, et al. An update on adult forms of hereditary pheochromocytomas and paragangliomas. Curr Opin Oncol. 2021 Jan;33(1):23-32. Abstract

23. Buffet A, Burnichon N, Favier J, et al. An overview of 20 years of genetic studies in pheochromocytoma and paraganglioma. Best Pract Res Clin Endocrinol Metab. 2020 Mar;34(2):101416.Full text  Abstract

24. Wachtel H, Fishbein L. Genetics of pheochromocytoma and paraganglioma. Curr Opin Endocrinol Diabetes Obes. 2021 Jun 1;28(3):283-90. Abstract

25. Alrezk R, Suarez A, Tena I, et al. Update of pheochromocytoma syndromes: genetics, biochemical evaluation, and imaging. Front Endocrinol (Lausanne). 2018 Nov 27:9:515.Full text  Abstract

26. Patel D, Phay JE, Yen TWF, et al. Update on pheochromocytoma and paraganglioma from the SSO Endocrine/Head and Neck Disease-Site Work Group. Part 1 of 2: Advances in pathogenesis and diagnosis of pheochromocytoma and paraganglioma. Ann Surg Oncol. 2020 May;27(5):1329-37.Full text  Abstract

27. Granberg D, Juhlin CC, Falhammar H. Metastatic pheochromocytomas and abdominal paragangliomas. J Clin Endocrinol Metab. 2021 Apr 23;106(5):e1937-52.Full text  Abstract

28. Fishbein L, Del Rivero J, Else T, et al. The North American Neuroendocrine Tumor Society consensus guidelines for surveillance and management of metastatic and/or unresectable pheochromocytoma and paraganglioma. Pancreas. 2021 Apr 1;50(4):469-93.Full text  Abstract

29. Marini F, Falchetti A, Del Monte F, et al. Multiple endocrine neoplasia type 2. Orphanet J Rare Dis. 2006 Nov 14;1:45.Full text  Abstract

30. Pomares FJ, Canas R, Rodriguez JM, et al. Differences between sporadic and multiple endocrine neoplasia type 2A phaeochromocytoma. Clin Endocrinol (Oxf). 1998 Feb;48(2):195-200. Abstract

31. Amodru V, Taieb D, Guerin C, et al. MEN2-related pheochromocytoma: current state of knowledge, specific characteristics in MEN2B, and perspectives. Endocrine. 2020 Sep;69(3):496-503. Abstract

32. Maher ER. Von Hippel-Lindau disease. Curr Mol Med. 2004 Dec;4(8):833-42. Abstract

33. Castro-Teles J, Sousa-Pinto B, Rebelo S, et al. Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: not a needle in a haystack. Endocr Connect. 2021 Oct 27;10(11):R293-304.Full text  Abstract

34. Louise M Binderup M, Smerdel M, Borgwadt L, et al. von Hippel-Lindau disease: updated guideline for diagnosis and surveillance. Eur J Med Genet. 2022 Aug;65(8):104538.Full text  Abstract

35. Hudler P, Urbancic M. The role of VHL in the development of von Hippel-Lindau disease and erythrocytosis. Genes (Basel). 2022 Feb 17;13(2):362.Full text  Abstract

36. Amar L, Pacak K, Steichen O, et al. International consensus on initial screening and follow-up of asymptomatic SDHx mutation carriers. Nat Rev Endocrinol. 2021 Jul;17(7):435-44.Full text  Abstract

37. Andrews KA, Ascher DB, Pires DEV, et al. Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD. J Med Genet. 2018 Jun;55(6):384-94.Full text  Abstract

38. Astuti D, Douglas F, Lennard TW, et al. Germline SDHD mutation in familial phaeochromocytoma. Lancet. 2001 Apr 14;357(9263):1181-2. Abstract

39. Sen I, Young WF Jr, Kasperbauer JL, et al. Tumor-specific prognosis of mutation-positive patients with head and neck paragangliomas. J Vasc Surg. 2020 May;71(5):1602-12.e2.Full text  Abstract

40. Walther MM, Herring J, Enquist E, et al. von Recklinghausen's disease and pheochromocytomas. J Urol. 1999 Nov;162(5):1582-6. Abstract

41. Zinnamosca L, Petramala L, Cotesta D, et al. Neurofibromatosis type 1 (NF1) and pheochromocytoma: prevalence, clinical and cardiovascular aspects. Arch Dermatol Res. 2011 Jul;303(5):317-25. Abstract

42. Gruber LM, Erickson D, Babovic-Vuksanovic D, et al. Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1. Clin Endocrinol (Oxf). 2017 Jan;86(1):141-9. Abstract

43. Shinall MC, Solórzano CC. Pheochromocytoma in neurofibromatosis type 1: when should it be suspected? Endocr Pract. 2014 Aug;20(8):792-6. Abstract

44. Rogowski-Lehmann N, Geroula A, Prejbisz A, et al. Missed clinical clues in patients with pheochromocytoma/paraganglioma discovered by imaging. Endocr Connect. 2018 Sep 1;7(11):1168-77.Full text  Abstract

45. Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175(2):G1-G34.Full text  Abstract

46. Zeiger MA, Thompson GB, Duh QY, et al. American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons medical guidelines for the management of adrenal incidentalomas: executive summary of recommendations. Endocr Pract. 2009 Jul-Aug;15(5):450-3. Abstract

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

48. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

49. Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Full text  Abstract

50. Remde H, Pamporaki C, Quinkler M, et al. Improved diagnostic accuracy of clonidine suppression testing using an age-related cutoff for plasma normetanephrine. Hypertension. 2022 Jun;79(6):1257-64.Full text  Abstract

51. Algeciras-Schimnich A, Preissner CM, Young WF Jr, et al. Plasma chromogranin A or urine fractionated metanephrines follow-up testing improves the diagnostic accuracy of plasma fractionated metanephrines for pheochromocytoma. J Clin Endocrinol Metab. 2008 Jan;93(1):91-5.Full text  Abstract

52. Timmers HJLM, Taïeb D, Pacak K, et al. Imaging of pheochromocytomas and paragangliomas. Endocr Rev. 2024 May 7;45(3):414-34.Full text  Abstract

53. American College of Radiology. ACR appropriateness criteria: adrenal mass evaluation. 2021 [internet publication].​Full text

54. Janssen I, Blanchet EM, Adams K, 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 Sep 1;21(17):3888-95.Full text  Abstract

55. Plouin PF, Amar L, Dekkers OM, et al; Guideline Working Group. 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 May;174(5):G1-10.Full text  Abstract

56. Manger WM, Gifford RW. Pheochromocytoma. J Clin Hypertens (Greenwich). 2002 Jan-Feb;4(1):62-72. Abstract

57. Drenou B, Le Tulzo Y, Caulet-Maugendre S, et al. Pheochromocytoma and secondary erythrocytosis: role of tumor erythropoietin secretion. Nouv Rev Fr Hematol. 1995;37(3):197-9. Abstract

58. Grossrubatscher E, Dalino P, Vignati F, et al. The role of chromogranin A in the management of patients with pheochromocytoma. Clin Endocrinol. 2006 Sep;65(3):287-93. Abstract

59. Sjoberg RJ, Simcic KJ, Kidd GS. The clonidine suppression test for pheochromocytoma: a review of its utility and pitfalls. Arch Intern Med. 1992 Jun;152(6):1193-7. Abstract

60. Fagundes GFC, Almeida MQ. Pitfalls in the diagnostic evaluation of pheochromocytomas. J Endocr Soc. 2024 Apr 6;8(6):bvae078.Full text  Abstract

61. Sinnott B, Hatipoglu B. Adrenal incidentaloma in pregnancy. Endocrinologist. 2005 Jul/Aug;15(4):205-8.

62. Sarathi VL, Lila AR, Bandgar TR, et al. Pheochromocytoma and pregnancy: a rare but dangerous combination. Endocr Pract. 2010 Mar-Apr;16(2):300-9. Abstract

63. Nazari MA, Hasan R, Haigney M, et al. Catecholamine-induced hypertensive crises: current insights and management. Lancet Diabetes Endocrinol. 2023 Dec;11(12):942-54. Abstract

64. Nicholson JP Jr, Vaughn ED Jr, Pickering TG, et al. Pheochromocytoma and prazosin. Ann Int Med. 1983 Oct;99(4):477-9. Abstract

65. National Center for Biotechnology Information. PubChem: metyrosine. Apr 2025 [internet publication].Full text

66. Tada K, Okuda T, Yamashita K. Three cases of malignant pheochromocytoma treated with cyclophosphamide, vincristine and dacarbazine combination chemotherapty and alpha-methyl-p-tyrosine to control hypercatecholaminemia. Horm Res. 1998;49(6):295-7. Abstract

67. Patel D, Phay JE, Yen TWF, et al. Update on pheochromocytoma and paraganglioma from the SSO Endocrine and Head and Neck Disease Site Working Group, part 2 of 2: perioperative management and outcomes of pheochromocytoma and paraganglioma. Ann Surg Oncol. 2020 May;27(5):1338-47.Full text  Abstract

68. Yip L, Duh QY, Wachtel H, et al. American Association of Endocrine Surgeons guidelines for adrenalectomy: executive summary. JAMA Surg. 2022 Oct 1;157(10):870-7.Full text  Abstract

69. Hue JJ, Alvarado C, Bachman K, et al. Outcomes of malignant pheochromocytoma based on operative approach: a National Cancer Database analysis. Surgery. 2021 Oct;170(4):1093-8. Abstract

70. Petri BJ, van Eijck CH, de Herder WW, et al. Phaeochromocytomas and sympathetic paragangliomas. Br J Surg. 2009 Dec;96(12):1381-92.Full text  Abstract

71. Jimenez C, Xu G, Varghese J, et al. New directions in treatment of metastatic or advanced pheochromocytomas and sympathetic paragangliomas: an American, contemporary, pragmatic approach. Curr Oncol Rep. 2022 Jan;24(1):89-98. Abstract

72. Tena I, Gupta G, Tajahuerce M, et al. Successful second-line metronomic temozolomide in metastatic paraganglioma: case reports and review of the literature. Clin Med Insights Oncol. 2018 Apr 9:12:1179554918763367.Full text  Abstract

73. Tong A, Li M, Cui Y, et al. Temozolomide is a potential therapeutic tool for patients with metastatic pheochromocytoma/paraganglioma-case report and review of the literature. Front Endocrinol (Lausanne). 2020 Feb 18:11:61.Full text  Abstract

74. Breen W, Bancos I, Young WF Jr, et al. External beam radiation therapy for advanced/unresectable malignant paraganglioma and pheochromocytoma. Adv Radiat Oncol. 2017 Nov 22;3(1):25-9.Full text  Abstract

75. Fishbein L, Bonner L, Torigian DA, 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 May;44(5):405-10. Abstract

76. Baudin E, Goichot B, Berruti A, et al. Sunitinib for metastatic progressive phaeochromocytomas and paragangliomas: results from FIRSTMAPPP, an academic, multicentre, international, randomised, placebo-controlled, double-blind, phase 2 trial. Lancet. 2024 Mar 16;403(10431):1061-70. Abstract

77. Pacak K, Fojo T, Goldstein DS, et al. Radiofrequency ablation: a novel approach for treatment of metastatic pheochromocytoma. J Natl Cancer Inst. 2001 Apr 18;93(8):648-9.Full text  Abstract

78. Yip L, Lee JE, Shapiro SE. Surgical management of hereditary pheochromocytoma. J Am Coll Surg. 2004 Apr;198(4):525-34. Abstract

79. Plouin PF, Duclos JM, Soppelsa F, et al. Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center. J Clin Endocrinol Metab. 2001 Apr;86(4):1480-6.Full text  Abstract

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

81. Akiba M, Kodama T, Ito Y, et al. Hypoglycemia induced by excessive rebound secretion of insulin after removal of pheochromocytoma. World J Surg. 1990 May-Jun;14(3):317-24. Abstract

82. Radtke WE, Kazmier FJ, Rutherford BD, et al. Cardiovascular complications of pheochromocytoma crisis. Am J Cardiol. 1975 May;35(5):701-5. Abstract

83. Steinsapir J, Carr AA, Prisant LM, et al. Metyrosine and pheochromocytoma. Arch Intern Med. 1997 Apr 28;157(8):901-6. Abstract

84. Pang Y, Liu Y, Pacak K, et al. Pheochromocytomas and paragangliomas: from genetic diversity to targeted therapies. Cancers (Basel). 2019 Mar 28;11(4):436.Full text  Abstract

85. Winzeler B, Challis BG, Casey RT. Precision medicine in phaeochromocytoma and paraganglioma. J Pers Med. 2021 Nov 22;11(11):1239.Full text  Abstract

86. Giubellino A, Sourbier C, Lee MJ, et al. Targeting heat shock protein 90 for the treatment of malignant pheochromocytoma. PLoS One. 2013;8(2):e56083.Full text  Abstract

87. Amar L, Lussey-Lepoutre C, Lenders JW, et al. Management of endocrine disease: recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: a systematic review and meta-analysis. Eur J Endocrinol. 2016 Oct;175(4):R135-45. Abstract

88. Fung MM, Viveros OH, O'Connor DT. Diseases of the adrenal medulla. Acta Physiol (Oxf). 2008 Feb;192(2):325-35. Abstract

89. Hamidi O, Young WF Jr, Gruber L, et al. Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: A systematic review and meta-analysis. Clin Endocrinol (Oxf). 2017 Nov;87(5):440-50. Abstract

90. Yoshida S, Hatori M, Noshiro T, et al. Twenty-six-years' survival with multiple bone metastasis of malignant pheochromocytoma. Arch Orthop Trauma Surg. 2001 Nov;121(10):598-600. Abstract

91. Mei L, Khurana A, Al-Juhaishi T, et al. Prognostic factors of malignant pheochromocytoma and paraganglioma: a combined SEER and TCGA databases review. Horm Metab Res. 2019 Jul;51(7):451-7. Abstract

92. Nölting S, Ullrich M, Pietzsch J, et al. Current management of pheochromocytoma/paraganglioma: a guide for the practicing clinician in the era of precision medicine. Cancers (Basel). 2019 Oct 8;11(10):1505.Full text  Abstract

93. Tan SG, Koay CK, Chan ST. The use of vasopressin to treat catecholamine-resistant hypotension after pheochromocyoma removal. Anaesth Intensive Care. 2002 Aug;30(4):477-80. Abstract

94. Rana HQ, Rainville IR, Vaidya A. Genetic testing in the clinical care of patients with pheochromocytoma and paraganglioma. Curr Opin Endocrinol Diabetes Obes. 2014 Jun;21(3):166-76. Abstract

95. Hampel H, Bennett RL, Buchanan A, et al. A practice guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: referral indications for cancer predisposition assessment. Genet Med. 2015 Jan;17(1):70-87.Full text  Abstract

96. Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002 Mar 20;287(11):1427-34.Full text  Abstract

97. Huang KH, Chung SD, Chen SC, et al. Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institution. Int J Urol. 2007 Mar;14(3):181-5. Abstract