Skip to main content

Surgical Versus Nonsurgical Management of Malignant Pheochromocytoma

  • Chapter
  • First Online:
Difficult Decisions in Endocrine Surgery

Part of the book series: Difficult Decisions in Surgery: An Evidence-Based Approach ((DDSURGERY))

  • 684 Accesses

Abstract

Malignant pheochromocytomas and paragangliomas are rare. Resection of the primary tumor and metastatic lesions, when feasible, is recommended. Goals of resection include improvement of clinical symptoms, reduction of catecholamine excess, local disease control, improved efficacy of subsequent non-operative therapies, and the possibility of improved survival. A R0 or R1 resection clearly provides more robust biochemical improvement when compared to a R2 resection or ‘surgical debulking.’ Additional non-surgical therapies can be used in conjunction with surgery or as the primary treatment modality in some cases. Non-surgical local therapies include external beam radiation, percutaneous tumor ablation, and directed transarterial chemoembolization. In addition, systemic therapies include radioactive iodine meta-iodobenzylguanidine (131I-MIBG), cytotoxic chemotherapy, and molecular targeted therapy. Care should be taken to provide patients with the appropriate pharmacologic adrenergic blockade prior to the initiation of most therapies. Patients benefit from a multidisciplinary approach at a center familiar with managing malignant pheochromocytoma patients.

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

Access this chapter

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
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

Similar content being viewed by others

References

  1. Fränkel F. Ein fall von doppelseitigen vollig latent verlaufen nebennierentumor und gleichseitiger nephritis mit veranderungen am circulation sappart und retinitis. Virchows Arch A. 1886;103:244.

    Article  Google Scholar 

  2. Pederson LC, Lee JE. Pheochromocytoma: Current Treat Options. Oncology. 2003;4(4):329–37.

    PubMed  Google Scholar 

  3. Tanka S, Ito T, Tomoda J, Higashi T, Yamada G, Tsuji T. Malignant pheochromocytoma with hepatic metastasis diagnosed 20 years after resection of the primary adrenal lesion. Intern Med. 1993;32:789–94.

    Article  Google Scholar 

  4. Ellis RJ, Patel D, Prodanov T, Sadowski S, Nilubol N, Adams K, Steinberg SM, Pacak K, Kebebew E. 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 Central  PubMed  Google Scholar 

  5. Khorram-Manesh A, Ahlman H, Nilsson O, Friberg P, Odén A, Stenström G, Hansson G, Stenquist O, Wängberg B, Tisell LE, Jansson S, Khorram-Manesh A, Ahlman H, Nilsson O, Friberg P, Odén A, Stenström G, Hansson G, Stenquist O, Wängberg B, Tisell LE, Jansson S. Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med. 2005;258(1):55–66.

    Article  CAS  PubMed  Google Scholar 

  6. Wan WH, Tan KY, Ng C, Tay KH, Mancer K, Tay MH, Chia WK, Soo KC, Ooi LL. Metastatic malignant phaeochromocytoma: a rare entity that underlies a therapeutic quandary. Asian J Surg. 2006;29(4):294–302.

    Article  PubMed  Google Scholar 

  7. Noda T, Nagano H, Miyamoto A, Wada H, Murakami M, Kobayashi S, Marubashi S, Takeda Y, Dono K, Umeshita K, Wakasa K, Monden M. Successful outcome after resection of liver metastasis arising from an extraadrenal retroperitoneal paraganglioma that appeared 9 years after surgical excision of the primary lesion. Int J Clin Oncol. 2009;14(5):473–7.

    Article  PubMed  Google Scholar 

  8. Mishra AK, Agarwal G, Kapoor A, Agarwal A, Bhatia E, Mishra SK. Catecholamine cardiomyopathy in bilateral malignant pheochromocytoma: successful reversal after surgery. Int J Cardiol. 2000;76(1):89–90.

    Article  CAS  PubMed  Google Scholar 

  9. Proye C, Vix M, Goropoulos A, Kerlo P, Lecomte-Houcke M. High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991. J Endocrinol Investig. 1992;15(9):651–63.

    Article  CAS  Google Scholar 

  10. Mahoney EM, Harrison JH. Malignant pheochromocytoma: clinical course and treatment. J Urol. 1977;118(2):225–9.

    Article  CAS  PubMed  Google Scholar 

  11. Guo JZ, Gong LS, Chen SX, Luo BY, Xu MY. Malignant pheochromocytoma: diagnosis and treatment in fifteen cases. J Hypertens. 1989;7:261–6.

    Article  CAS  PubMed  Google Scholar 

  12. Yoshida S, Hatori M, Noshiro T, Kimura N, Kokubun S. Twenty-six-years’ survival with multiple bone metastasis of malignant pheochromocytoma. Arch Orthop Trauma Surg. 2001;121:598–600.

    Article  CAS  PubMed  Google Scholar 

  13. Pacak K, Eisenhofer G, Ahlman H, et al. Pheochromocytoma: recommendations for clinical practice from the first international symposium. Nat Clin Pract Endocrinol Metab. 2007;3(2):92–102.

    Article  PubMed  Google Scholar 

  14. Huang KH, Chung SD, Chen SC, Chueh SC, Pu YS, Lai MK, Lin WC. Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute. Int J Urol. 2007;14(3):181–5.

    Article  CAS  PubMed  Google Scholar 

  15. Joseph L. Malignant pheochromocytoma of the organ of Zuckerkandl with functioning metastases. Br J Urol. 1967;39:221–5.

    Article  CAS  PubMed  Google Scholar 

  16. Brauckhoff M, Gimm O, Dralle H. Preoperative and surgical therapy in sporadic and familial pheochromocytoma. Front Horm Res. 2004;31:121–44.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  18. Pacak K, Ilias I, Adams KT, Eisenhofer G. Biochemical diagnosis, localization and management of pheochromocytoma: focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the toumour. J Intern Med. 2005;257:60–8.

    Article  CAS  PubMed  Google Scholar 

  19. van der Harst E, de Herder WW, Bruining HA, Bonjer HJ, de Krijger RR, Lamberts SW, van de Meiracker AH, Boomsma F, Stijnen T, Krenning EP, Bosman FT, Kwekkeboom DJ. [(123)I]metaiodobenzylguanidine and [(111)in]octreotide uptake in begnign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2001;86(2):685–93.

    PubMed  Google Scholar 

  20. 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 

  21. 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 962-3.

    Article  PubMed  Google Scholar 

  22. 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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  23. Baudin E, Habra MA, Deschamps F, Cote G, Dumont F, Cabanillas M, Arfi-Roufe J, Berdelou A, Moon B, Al Ghuzlan A, Patel S, Leboulleux S, Jimenez C. Therapy of endocrine disease: treatment of malignant pheochromocytoma and paraganglioma. Eur J Endocrinol. 2014;171(3):R111–22.

    Article  CAS  PubMed  Google Scholar 

  24. Fishbein L, Bonner L, Torigian DA, Nathanson KL, Cohen DL, Pryma D, Cengel KA. 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  PubMed Central  CAS  PubMed  Google Scholar 

  25. 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.

    Article  PubMed  Google Scholar 

  26. Hidaka S, Hiraoka A, Ochi H, Uehara T, Ninomiya T, Miyamoto Y, Hasebe A, Tanihira T, Tanabe A, Ichiryu M, Nakahara H, Tazuya N, Ninomiya I, Michitaka K. Malignant pheochromocytoma with liver metastasis treated by transcatheter arterial chemo-embolization (TACE). Intern Med. 2010;49(7):645–51.

    Article  PubMed  Google Scholar 

  27. Takahashi K, Ashizawa N, Minami T, Suzuki S, Sakamoto I, Hayashi K, Tomiyasu S, Sumikawa K, Kitamura K, Eto T, Yano K. Malignant pheochromocytoma with multiple hepatic metastases treated by chemotherapy and transcatheter arterial embolization. Intern Med. 1999;38(4):349–54.

    Article  CAS  PubMed  Google Scholar 

  28. Watanabe D, Tanabe A, Naruse M, Tsuiki M, Torii N, Noshiro T, Takano K. Transcatheter arterial embolization for the treatment of liver metastases in a patient with malignant pheochromocytoma. Endocr J. 2006;53(1):59–66.

    Article  PubMed  Google Scholar 

  29. 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 

  30. Ayala-Ramirez M, Feng L, Habra MA, Rich T, Dickson PV, Perrier N, Phan A, Waguespack S, Patel S, Jimenez C. 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 

  31. 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.

    Article  CAS  PubMed  Google Scholar 

  32. 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. Horm Metab Res. 2009;41(9):697–702.

    Article  CAS  PubMed  Google Scholar 

  33. 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.

    Article  CAS  PubMed  Google Scholar 

  34. 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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Cohen, M.S., Cotton, T.M. (2018). Surgical Versus Nonsurgical Management of Malignant Pheochromocytoma. In: Angelos, P., Grogan, R. (eds) Difficult Decisions in Endocrine Surgery. Difficult Decisions in Surgery: An Evidence-Based Approach. Springer, Cham. https://doi.org/10.1007/978-3-319-92860-9_29

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-92860-9_29

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-92858-6

  • Online ISBN: 978-3-319-92860-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics