open access

Ahead of print
Review paper
Published online: 2023-11-20
Get Citation

Advances in management of pheochromocytoma – a short review

Michał Miciak1, Krzysztof Jurkiewicz1, Krzysztof Kaliszewski1
Affiliations
  1. Department of General, Minimally Invasive and Endocrine Surgery Wroclaw Medical University, Wrocław, Poland

open access

Ahead of print
Review articles – Pheochromocytoma
Published online: 2023-11-20

Abstract

Pheochromocytoma is a rare neuroendocrine neoplasm. It is characterized by overproduction of catecholamines, which causes clinical symptoms associated with elevated blood pressure values and can even lead to life-threatening complications. The tumor can be associated with genetic syndromes such as MEN-2 or VHL, and currently available and constantly evolving genetic testing makes it possible to detect the inherited form and plan appropriate therapy. Management of pheochromocytoma is based on initial laboratory diagnosis, confirmation by imaging studies, determination of hormonal activity and resulting therapy. Surgical resection by laparoscopic approach is the most recommended. For unresectable tumor or advanced disease with distant metastases, systemic therapies developing all the time currently allow cure or inhibition of tumor progression. In this paper, we will review advances in management of pheochromocytoma over the past decade and potential directions for future research.

Abstract

Pheochromocytoma is a rare neuroendocrine neoplasm. It is characterized by overproduction of catecholamines, which causes clinical symptoms associated with elevated blood pressure values and can even lead to life-threatening complications. The tumor can be associated with genetic syndromes such as MEN-2 or VHL, and currently available and constantly evolving genetic testing makes it possible to detect the inherited form and plan appropriate therapy. Management of pheochromocytoma is based on initial laboratory diagnosis, confirmation by imaging studies, determination of hormonal activity and resulting therapy. Surgical resection by laparoscopic approach is the most recommended. For unresectable tumor or advanced disease with distant metastases, systemic therapies developing all the time currently allow cure or inhibition of tumor progression. In this paper, we will review advances in management of pheochromocytoma over the past decade and potential directions for future research.

Get Citation

Keywords

pheochromocytoma; management; imaging studies; systemic therapy; advances

About this article
Title

Advances in management of pheochromocytoma – a short review

Journal

Nowotwory. Journal of Oncology

Issue

Ahead of print

Article type

Review paper

Published online

2023-11-20

Page views

18

Article views/downloads

26

Keywords

pheochromocytoma
management
imaging studies
systemic therapy
advances

Authors

Michał Miciak
Krzysztof Jurkiewicz
Krzysztof Kaliszewski

References (55)
  1. Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014; 38(1): 7–41.
  2. Treiyer A, Janssen M, Kamradt J, et al. [Initial experience of a series of robotic-assisted laparoscopic adrenalectomy]. Actas Urol Esp. 2013; 37(1): 54–59.
  3. Conzo G, Pasquali D, Colantuoni V, et al. Current concepts of pheochromocytoma. Int J Surg. 2014; 12(5): 469–474.
  4. Gunawardane PT, Grossman A. Phaeochromocytoma and Paraganglioma. Adv Exp Med Biol. 2017; 956: 239–259.
  5. Ku EuJ, Kim KJ, Kim JH, et al. Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force. Endocrinol Metab (Seoul). 2021; 36(2): 322–338.
  6. Polanowski P, Kotecka-Blicharz A, Chmielik E, et al. Paragangliomas of the head and neck region. Nowotwory. Journal of Oncology. 2018; 68(3): 132–139.
  7. 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; 34(2): 101416.
  8. Ma X, Li M, Tong A, et al. Genetic and Clinical Profiles of Pheochromocytoma and Paraganglioma: A Single Center Study. Front Endocrinol (Lausanne). 2020; 11: 574662.
  9. Choi H, Kim KJ, Hong N, et al. Genetic Analysis and Clinical Characteristics of Hereditary Pheochromocytoma and Paraganglioma Syndrome in Korean Population. Endocrinol Metab (Seoul). 2020; 35(4): 858–872.
  10. 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; 8(11): 894–902.
  11. 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; 51: 68–73.
  12. Jain A, Baracco R, Kapur G. Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management. Pediatr Nephrol. 2020; 35(4): 581–594.
  13. Neumann HPH, Young WF, Eng C. Pheochromocytoma and Paraganglioma. N Engl J Med. 2019; 381(6): 552–565.
  14. Y-Hassan S, Falhammar H. Cardiovascular Manifestations and Complications of Pheochromocytomas and Paragangliomas. J Clin Med. 2020; 9(8).
  15. Zhao L, Meng Xu, Mei Q, et al. Risk Factors for Cardiac Complications in Patients With Pheochromocytoma and Paraganglioma: A Retrospective Single-Center Study. Front Endocrinol (Lausanne). 2022; 13: 877341.
  16. Foltyński N, Wawryka S. Zespół Wunderlicha z objawami wstrząsu septycznego jako pierwszy objaw raka jasnokomórkowego nerki — opis przypadku klinicznego. Nowotwory. Journal of Oncology. 2015; 65(2): 135–138.
  17. Norton JA, Krampitz G, Jensen RT. Multiple Endocrine Neoplasia: Genetics and Clinical Management. Surg Oncol Clin N Am. 2015; 24(4): 795–832.
  18. Parenti G, Zampetti B, Rapizzi E, et al. Updated and new perspectives on diagnosis, prognosis, and therapy of malignant pheochromocytoma/paraganglioma. J Oncol. 2012; 2012: 872713.
  19. Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, et al. Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas. Clin Transl Oncol. 2021; 23(10): 1995–2019.
  20. Eisenhofer G, Prejbisz A, Peitzsch M, et al. Biochemical Diagnosis of Chromaffin Cell Tumors in Patients at High and Low Risk of Disease: Plasma versus Urinary Free or Deconjugated -Methylated Catecholamine Metabolites. Clin Chem. 2018; 64(11): 1646–1656.
  21. Joyce CM, Melvin A, O'Shea PM, et al. Case report of a phantom pheochromocytoma. Biochem Med (Zagreb). 2020; 30(2): 021003.
  22. Casey R, Griffin TP, Wall D, et al. Screening for phaeochromocytoma and paraganglioma: impact of using supine reference intervals for plasma metanephrines with samples collected from fasted/seated patients. Ann Clin Biochem. 2017; 54(1): 170–173.
  23. Därr R, Kuhn M, Bode C, et al. Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review. Endocrine. 2017; 56(3): 495–503.
  24. Neary NM, King KS, Pacak K. Drugs and pheochromocytoma--don't be fooled by every elevated metanephrine. N Engl J Med. 2011; 364(23): 2268–2270.
  25. Bílek R, Vlček P, Šafařík L, et al. Chromogranin A in the Laboratory Diagnosis of Pheochromocytoma and Paraganglioma. Cancers (Basel). 2019; 11(4).
  26. Gut P, Czarnywojtek A, Fischbach J, et al. Chromogranin A - unspecific neuroendocrine marker. Clinical utility and potential diagnostic pitfalls. Arch Med Sci. 2016; 12(1): 1–9.
  27. El Lakis M, Gianakou A, Nockel P, et al. Radioguided Surgery With Gallium 68 Dotatate for Patients With Neuroendocrine Tumors. JAMA Surg. 2019; 154(1): 40–45.
  28. Mercado-Asis LB, Wolf KI, Jochmanova I, et al. PHEOCHROMOCYTOMA: A GENETIC AND DIAGNOSTIC UPDATE. Endocr Pract. 2018; 24(1): 78–90.
  29. Kiernan CM, Solórzano CC. Pheochromocytoma and Paraganglioma: Diagnosis, Genetics, and Treatment. Surg Oncol Clin N Am. 2016; 25(1): 119–138.
  30. Antonio K, Valdez MaM, Mercado-Asis L, et al. Pheochromocytoma/paraganglioma: recent updates in genetics, biochemistry, immunohistochemistry, metabolomics, imaging and therapeutic options. Gland Surg. 2020; 9(1): 105–123.
  31. Fishbein L, Leshchiner I, Walter V, et al. Cancer Genome Atlas Research Network. Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma. Cancer Cell. 2017; 31(2): 181–193.
  32. Białas M, Okoń K, Dyduch G, et al. Neuroendocrine markers and sustentacular cell count in benign and malignant pheochromocytomas - a comparative study. Pol J Pathol. 2013; 64(2): 129–135.
  33. Su T, Yang Y, Jiang L, et al. SDHB immunohistochemistry for prognosis of pheochromocytoma and paraganglioma: A retrospective and prospective analysis. Front Endocrinol (Lausanne). 2023; 14: 1121397.
  34. Chopko BW. Endovascular treatment of vertebral column metastases using intra-arterial Cisplatin: pilot experience. Case Rep Med. 2014; 2014: 915904.
  35. Biggar MA, Lennard TWJ. Systematic review of phaeochromocytoma in pregnancy. Br J Surg. 2013; 100(2): 182–190.
  36. Conzo G, Musella M, Corcione F, et al. Laparoscopic adrenalectomy, a safe procedure for pheochromocytoma. A retrospective review of clinical series. Int J Surg. 2013; 11(2): 152–156.
  37. Bednarczuk T, Bolanowski M, Sworczak K, et al. Adrenal incidentaloma in adults - management recommendations by the Polish Society of Endocrinology. Endokrynol Pol. 2016; 67(2): 234–258.
  38. De Filpo G, Maggi M, Mannelli M, et al. Management and outcome of metastatic pheochromocytomas/paragangliomas: an overview. J Endocrinol Invest. 2021; 44(1): 15–25.
  39. Amar L, Lussey-Lepoutre C, Lenders JWM, 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; 175(4): R135–R145.
  40. Ramakrishna H. Pheochromocytoma resection: Current concepts in anesthetic management. J Anaesthesiol Clin Pharmacol. 2015; 31(3): 317–323.
  41. Connor D, Boumphrey S. Perioperative care of phaeochromocytoma. BJA Education. 2016; 16(5): 153–158.
  42. Lowery AJ, Walsh S, McDermott EW, et al. Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas. Oncologist. 2013; 18(4): 391–407.
  43. Krishnappa R, Chikaraddi SB, Arun HN, et al. Pheochromocytoma in Indian patients: a retrospective study. Indian J Cancer. 2012; 49(1): 188–193.
  44. Nemoto K, Miura T, Shioji Go, et al. Sunitinib treatment for refractory malignant pheochromocytoma. Neuro Endocrinol Lett. 2012; 33(3): 260–264.
  45. Tang KL, Lin Yi, Li LM. Diagnosis and surgical treatment of multiple endocrine neoplasia type 2A. World J Surg Oncol. 2014; 12: 8.
  46. Deutschbein T, Fassnacht M, Weismann D, et al. Treatment of malignant phaeochromocytoma with a combination of cyclophosphamide, vincristine and dacarbazine: own experience and overview of the contemporary literature. Clin Endocrinol (Oxf). 2015; 82(1): 84–90.
  47. Ye YL, Yuan XX, Chen MK, et al. Management of adrenal incidentaloma: the role of adrenalectomy may be underestimated. BMC Surg. 2016; 16(1): 41.
  48. Ferrara AM, Lombardi G, Pambuku A, et al. Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma. Anticancer Drugs. 2018; 29(1): 102–105.
  49. Zhang X, Wang X, Qin L, et al. The dual mTORC1 and mTORC2 inhibitor PP242 shows strong antitumor activity in a pheochromocytoma PC12 cell tumor model. Urology. 2015; 85(1): 273.e1–273.e7.
  50. 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.
  51. Zhang X, Gao F, Zhong S. Combinatorial Inhibition of mTORC2 and Hsp90 Leads to a Distinctly Effective Therapeutic Strategy in Malignant Pheochromocytoma. Curr Cancer Drug Targets. 2019; 19(9): 698–706.
  52. Corssmit EPM, Snel M, Kapiteijn E. Malignant pheochromocytoma and paraganglioma: management options. Curr Opin Oncol. 2020; 32(1): 20–26.
  53. 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; 24(1): 89–98.
  54. Meireles CG, Lourenço de Lima C, Martins de Paula Oliveira M, et al. Antiproliferative effects of metformin in cellular models of pheochromocytoma. Mol Cell Endocrinol. 2022; 539: 111484.
  55. Tabebi M, Söderkvist P, Gimm O. Nuclear and mitochondrial DNA alterations in pheochromocytomas and paragangliomas, and their potential treatment. Endocr Relat Cancer. 2023; 30(1).

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Wydawcą serwisu jest VM Media Group sp. z o.o., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl