Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, characterized by a high morbidity rate due to catecholamine excess. These high levels are independent of physiologic stressors. For the diagnosis, a biochemical workup is paramount. The most widely used are plasma-free metanephrines and urinary fractionated metanephrines. Imaging studies should be initiated once the biochemical diagnosis is established. Evaluation of the patient with pheochromocytomas and paragangliomas must be done taking into account the leading causes of perioperative morbidity and mortality. The two primary interventions that have reduced perioperative mortality are alpha-adrenergic blockade and intravascular volume normalization. Another significant advance has been the establishment of laparoscopic surgery as the gold standard for the surgical approach. No anesthetic technique has been found to be superior to another. Intraoperative hemodynamic instability has been correlated with poorer outcomes; thus one of the main intraoperative goals is maintaining hemodynamic stability. Lower morbidity and almost zero mortality rates due to preoperative and intraoperative management improvements have led to a focus on the immediate and long-term postoperative care. Anual lifelong follow-up is recommended to detect recurrent disease.

Authors: Magdalena Fermandois[1], Jaime Altamirano[2], Felipe Rojas[1], Cristóbal Román[3], Iván Pinto[2]

Affiliation:
[1] Departamento de Anestesiología, Facultad de Medicina, Universidad de los Andes, Santiago, Chile
[2] Instituto Oncológico Fundación Arturo López Pérez, Santiago, Chile
[3] Departamento de Urología, Facultad de Medicina, Universidad del Desarrollo, Santiago, Chile

E-mail: fermandois@gmail.com

Author address:
[1] Cachapoal 7724
Las Condes
Santiago
Chile

Citation: Fermandois M, Altamirano J, Rojas F, Román C, Pinto I. . Medwave 2020;20(2):e7836 doi: 10.5867/medwave.2020.02.7836

Submission date: 15/8/2019

Acceptance date: 30/12/2019

Publication date: 4/3/2020

Origin: Not commissioned.

Type of review: Externally peer-reviewed by two reviewers, double-blind.

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1. 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 Jun;15(6):428-34. | CrossRef | PubMed |
2. 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 Jun;99(6):1915-42. | CrossRef | PubMed |
3. Fleisher LA. Anesthesia and Uncommon Diseases: Sixth Edition. Anesthesia and Uncommon Diseases: Sixth Edition. 2012; 424-426. | CrossRef |
4. Wein AJ, Kavoussi LR, Partin AW, Peters CA. Campbell-Walsh Urology 11th Edition. Elsevier. 2015; 1528–1595.
5. 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 Feb;185(1):43-9. | CrossRef | PubMed |
6. Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. 2007 Feb 8;356(6):601-10. | PubMed |
7. Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, 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. | CrossRef | PubMed |
8. Brunaud L, Nguyen-Thi PL, Mirallie E, Raffaelli M, Vriens M, Theveniaud PE, et al. Predictive factors for postoperative morbidity after laparoscopic adrenalectomy for pheochromocytoma: a multicenter retrospective analysis in 225 patients. Surg Endosc. 2016 Mar;30(3):1051-9. | CrossRef | PubMed |
9. Farrugia FA, Martikos G, Surgeon C, Tzanetis P, Misiakos E, Zavras N, et al. Radiology of the adrenal incidentalomas. Review of the literature. Endocr Regul. 2017 Jan 1;51(1):35-51. | CrossRef | PubMed |
10. Maurea S, Klain M, Mainolfi C, Ziviello M, Salvatore M. The diagnostic role of radionuclide imaging in evaluation of patients with nonhypersecreting adrenal masses. J Nucl Med. 2001 Jun;42(6):884-92. | PubMed |
11. Boland GW, Blake MA, Hahn PF, Mayo-Smith WW. Incidental adrenal lesions: principles, techniques, and algorithms for imaging characterization. Radiology. 2008 Dec;249(3):756-75. | CrossRef | PubMed |
12. Kim SJ, Lee SW, Pak K, Kim IJ, Kim K. Diagnostic accuracy of (18)F-FDG PET or PET/CT for the characterization of adrenal masses: a systematic review and meta-analysis. Br J Radiol. 2018 Jun;91(1086):20170520. | CrossRef | PubMed |
13. Boland GW, Dwamena BA, Jagtiani Sangwaiya M, Goehler AG, Blake MA, et al. Characterization of adrenal masses by using FDG PET: a systematic review and meta-analysis of diagnostic test performance. Radiology. 2011 Apr;259(1):117-26. | CrossRef | PubMed |
14. Ramakrishna H. Pheochromocytoma resection: Current concepts in anesthetic management. J Anaesthesiol Clin Pharmacol. 2015 Jul-Sep;31(3):317-23. | CrossRef | PubMed |
15. Kristensen SD, Knuuti J, Saraste A, Anker S, Bøtker HE, Hert SD, et al. 2014 ESC/ESA Guidelines on non-cardiac surgery: cardiovascular assessment and management: The Joint Task Force on non-cardiac surgery: cardiovascular assessment and management of the European Society of Cardiology (ESC) and the European Society of Anaesthesiology (ESA). Eur Heart J. 2014 Sep 14;35(35):2383-431. | CrossRef | PubMed |
16. Fleisher LA, Fleischmann KE, Auerbach AD, Barnason SA, Beckman JA, Bozkurt B, et al. 2014 ACC/AHA guideline on perioperative cardiovascular evaluation and management of patients undergoing noncardiac surgery: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014 Dec 9;130(24):e278-333. | CrossRef | PubMed |
17. Roizen MF, Schreider BD, Hassan SZ. Anesthesia for patients with pheochromocytoma. Anesthesiol Clin North America. 1987;5:269-75.
18. Smith J, Howards S, Preminger G, Dmochowski R. Hinman's Atlas of Urologic Surgery 4th Edition. Elsevier. 2012; 1097-1101.
19. Thornton JK. Abdominal nephrectomy for large sarcoma of the left suprarenal capsule: recovery. Trans Clin Soc Lond. 1890;23:150–3.
20. Mayo CH. Paroxysmal hypertension with tumor of retroperitoneal nerve: report of case. J Am Med Assoc. 1927;89:1047-1050
21. Gagner M, Lacroix A, Bolté E. Laparoscopic adrenalectomy in Cushing's syndrome and pheochromocytoma. N Engl J Med. 1992 Oct 1;327(14):1033. | PubMed |
22. Piazza L, Caragliano P, Scardilli M, Sgroi AV, Marino G, Giannone G. Laparoscopic robot-assisted right adrenalectomy and left ovariectomy (case reports). Chir Ital. 1999 Nov-Dec;51(6):465-6. | PubMed |
23. Porpiglia F, Miller BS, Manfredi M, Fiori C, Doherty GM. A debate on laparoscopic versus open adrenalectomy for adrenocortical carcinoma. Horm Cancer. 2011 Dec;2(6):372-7. | CrossRef | PubMed |
24. Brunt LM. The positive impact of laparoscopic adrenalectomy on complications of adrenal surgery. Surg Endosc. 2002 Feb;16(2):252-7. | PubMed |
25. Lee J, El-Tamer M, Schifftner T, Turrentine FE, Henderson WG, Khuri S, et al. Open and laparoscopic adrenalectomy: analysis of the National Surgical Quality Improvement Program. J Am Coll Surg. 2008 May;206(5):953-9; discussion 959-61. | CrossRef | PubMed |
26. Arezzo A, Bullano A, Cochetti G, Cirocchi R, Randolph J, Mearini E, et al. Transperitoneal versus retroperitoneal laparoscopic adrenalectomy for adrenal tumours in adults. Cochrane Database Syst Rev. 2018 Dec 30;12:CD011668. | CrossRef | PubMed |
27. Morino M, Benincà G, Giraudo G, Del Genio GM, Rebecchi F, Garrone C. Robot-assisted vs laparoscopic adrenalectomy: a prospective randomized controlled trial. Surg Endosc. 2004 Dec;18(12):1742-6. | PubMed |
28. Brunaud L, Bresler L, Zarnegar R, Ayav A, Cormier L, Tretou S, et al. Does robotic adrenalectomy improve patient quality of life when compared to laparoscopic adrenalectomy? World J Surg. 2004 Nov;28(11):1180-5. | PubMed |
29. Castillo O, Cortés O, Kerkebe M, Pinto P, ArellanoL, Russo M. Adrenalectomía laparoscópica: lecciones aprendidas en 110 procedimientos consecutivos. Rev Chil Cir. 2006;58(3):175-180.

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 Jun;15(6):428-34. | CrossRef | PubMed |

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 Jun;99(6):1915-42. | CrossRef | PubMed |

Fleisher LA. Anesthesia and Uncommon Diseases: Sixth Edition. Anesthesia and Uncommon Diseases: Sixth Edition. 2012; 424-426. | CrossRef |

Wein AJ, Kavoussi LR, Partin AW, Peters CA. Campbell-Walsh Urology 11th Edition. Elsevier. 2015; 1528–1595.

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 Feb;185(1):43-9. | CrossRef | PubMed |

Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. 2007 Feb 8;356(6):601-10. | PubMed |

Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, 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. | CrossRef | PubMed |

Brunaud L, Nguyen-Thi PL, Mirallie E, Raffaelli M, Vriens M, Theveniaud PE, et al. Predictive factors for postoperative morbidity after laparoscopic adrenalectomy for pheochromocytoma: a multicenter retrospective analysis in 225 patients. Surg Endosc. 2016 Mar;30(3):1051-9. | CrossRef | PubMed |

Farrugia FA, Martikos G, Surgeon C, Tzanetis P, Misiakos E, Zavras N, et al. Radiology of the adrenal incidentalomas. Review of the literature. Endocr Regul. 2017 Jan 1;51(1):35-51. | CrossRef | PubMed |

Maurea S, Klain M, Mainolfi C, Ziviello M, Salvatore M. The diagnostic role of radionuclide imaging in evaluation of patients with nonhypersecreting adrenal masses. J Nucl Med. 2001 Jun;42(6):884-92. | PubMed |

Boland GW, Blake MA, Hahn PF, Mayo-Smith WW. Incidental adrenal lesions: principles, techniques, and algorithms for imaging characterization. Radiology. 2008 Dec;249(3):756-75. | CrossRef | PubMed |

Kim SJ, Lee SW, Pak K, Kim IJ, Kim K. Diagnostic accuracy of (18)F-FDG PET or PET/CT for the characterization of adrenal masses: a systematic review and meta-analysis. Br J Radiol. 2018 Jun;91(1086):20170520. | CrossRef | PubMed |

Boland GW, Dwamena BA, Jagtiani Sangwaiya M, Goehler AG, Blake MA, et al. Characterization of adrenal masses by using FDG PET: a systematic review and meta-analysis of diagnostic test performance. Radiology. 2011 Apr;259(1):117-26. | CrossRef | PubMed |

Ramakrishna H. Pheochromocytoma resection: Current concepts in anesthetic management. J Anaesthesiol Clin Pharmacol. 2015 Jul-Sep;31(3):317-23. | CrossRef | PubMed |

Kristensen SD, Knuuti J, Saraste A, Anker S, Bøtker HE, Hert SD, et al. 2014 ESC/ESA Guidelines on non-cardiac surgery: cardiovascular assessment and management: The Joint Task Force on non-cardiac surgery: cardiovascular assessment and management of the European Society of Cardiology (ESC) and the European Society of Anaesthesiology (ESA). Eur Heart J. 2014 Sep 14;35(35):2383-431. | CrossRef | PubMed |

Fleisher LA, Fleischmann KE, Auerbach AD, Barnason SA, Beckman JA, Bozkurt B, et al. 2014 ACC/AHA guideline on perioperative cardiovascular evaluation and management of patients undergoing noncardiac surgery: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014 Dec 9;130(24):e278-333. | CrossRef | PubMed |

Roizen MF, Schreider BD, Hassan SZ. Anesthesia for patients with pheochromocytoma. Anesthesiol Clin North America. 1987;5:269-75.

Smith J, Howards S, Preminger G, Dmochowski R. Hinman's Atlas of Urologic Surgery 4th Edition. Elsevier. 2012; 1097-1101.

Thornton JK. Abdominal nephrectomy for large sarcoma of the left suprarenal capsule: recovery. Trans Clin Soc Lond. 1890;23:150–3.

Mayo CH. Paroxysmal hypertension with tumor of retroperitoneal nerve: report of case. J Am Med Assoc. 1927;89:1047-1050

Gagner M, Lacroix A, Bolté E. Laparoscopic adrenalectomy in Cushing's syndrome and pheochromocytoma. N Engl J Med. 1992 Oct 1;327(14):1033. | PubMed |

Piazza L, Caragliano P, Scardilli M, Sgroi AV, Marino G, Giannone G. Laparoscopic robot-assisted right adrenalectomy and left ovariectomy (case reports). Chir Ital. 1999 Nov-Dec;51(6):465-6. | PubMed |

Porpiglia F, Miller BS, Manfredi M, Fiori C, Doherty GM. A debate on laparoscopic versus open adrenalectomy for adrenocortical carcinoma. Horm Cancer. 2011 Dec;2(6):372-7. | CrossRef | PubMed |

Brunt LM. The positive impact of laparoscopic adrenalectomy on complications of adrenal surgery. Surg Endosc. 2002 Feb;16(2):252-7. | PubMed |

Lee J, El-Tamer M, Schifftner T, Turrentine FE, Henderson WG, Khuri S, et al. Open and laparoscopic adrenalectomy: analysis of the National Surgical Quality Improvement Program. J Am Coll Surg. 2008 May;206(5):953-9; discussion 959-61. | CrossRef | PubMed |

Arezzo A, Bullano A, Cochetti G, Cirocchi R, Randolph J, Mearini E, et al. Transperitoneal versus retroperitoneal laparoscopic adrenalectomy for adrenal tumours in adults. Cochrane Database Syst Rev. 2018 Dec 30;12:CD011668. | CrossRef | PubMed |

Morino M, Benincà G, Giraudo G, Del Genio GM, Rebecchi F, Garrone C. Robot-assisted vs laparoscopic adrenalectomy: a prospective randomized controlled trial. Surg Endosc. 2004 Dec;18(12):1742-6. | PubMed |

Brunaud L, Bresler L, Zarnegar R, Ayav A, Cormier L, Tretou S, et al. Does robotic adrenalectomy improve patient quality of life when compared to laparoscopic adrenalectomy? World J Surg. 2004 Nov;28(11):1180-5. | PubMed |

Castillo O, Cortés O, Kerkebe M, Pinto P, ArellanoL, Russo M. Adrenalectomía laparoscópica: lecciones aprendidas en 110 procedimientos consecutivos. Rev Chil Cir. 2006;58(3):175-180.