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Surgical Versus Nonsurgical Management of Malignant Pheochromocytoma

  • Mark S. Cohen
  • Travis M. Cotton
Chapter
Part of the Difficult Decisions in Surgery: An Evidence-Based Approach book series (DDSURGERY)

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.

Keywords

Malignant pheochromocytoma Metastatic pheochromocytoma Pheochromocytoma 131I-MIBG External beam radiation therapy Percutaneous tumor ablation Transarterial chemoembolization Cytotoxic chemotherapy Targeted molecular therapy 

References

  1. 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.CrossRefGoogle Scholar
  2. 2.
    Pederson LC, Lee JE. Pheochromocytoma: Current Treat Options. Oncology. 2003;4(4):329–37.PubMedPubMedCentralGoogle Scholar
  3. 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.CrossRefGoogle Scholar
  4. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  5. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  6. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  7. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  8. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  9. 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.CrossRefGoogle Scholar
  10. 10.
    Mahoney EM, Harrison JH. Malignant pheochromocytoma: clinical course and treatment. J Urol. 1977;118(2):225–9.CrossRefPubMedCentralPubMedGoogle Scholar
  11. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  12. 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.CrossRefPubMedGoogle Scholar
  13. 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.CrossRefPubMedGoogle Scholar
  14. 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.CrossRefPubMedGoogle Scholar
  15. 15.
    Joseph L. Malignant pheochromocytoma of the organ of Zuckerkandl with functioning metastases. Br J Urol. 1967;39:221–5.CrossRefPubMedGoogle Scholar
  16. 16.
    Brauckhoff M, Gimm O, Dralle H. Preoperative and surgical therapy in sporadic and familial pheochromocytoma. Front Horm Res. 2004;31:121–44.CrossRefPubMedGoogle Scholar
  17. 17.
    Adjallé R, Plouin PF, Pacak K, Lehnert H. Treatment of malignant pheochromocytoma. Horm Metab Res. 2009;41(9):687–96.CrossRefPubMedGoogle Scholar
  18. 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.CrossRefPubMedGoogle Scholar
  19. 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.PubMedGoogle Scholar
  20. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  21. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  22. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  23. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  24. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  25. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  26. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  27. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  28. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  29. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  30. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  31. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  32. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  33. 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.CrossRefPubMedCentralPubMedGoogle Scholar
  34. 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.CrossRefPubMedCentralPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Mark S. Cohen
    • 2
  • Travis M. Cotton
    • 1
  1. 1.Department of Surgery, Division of Endocrine SurgeryTaubman CenterAnn ArborUSA
  2. 2.Department of Endocrine SurgeryWarren Alpert Medical School of Brown UniversityProvidenceUSA

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