Skip to main content
SpringerLink
Log in
Book cover

Difficult Decisions in Endocrine Surgery pp 375–393Cite as

Surgery Versus Nonsurgical Therapy for Recurrent Adrenocortical Carcinoma

  • Chapter
  • First Online:

  • 657 Accesses

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

Abstract

Adrenocortical carcinoma is a rare endocrine neoplasm associated with poor prognosis. Complete surgical resection is the only potential cure for the disease. Unfortunately, a significant number of patients develop disease relapse and present with local or systemic recurrence. Close follow-up with regular clinical examination aided by radiological imaging and blood investigations is crucial for the early detection of recurrent disease. The best treatment options for recurrent disease remain unclear and these include surgery, chemotherapy, and radiotherapy, in addition to new and upcoming treatments. This chapter will focus on the treatment of recurrent ACC including a discussion of surgical and non-surgical therapy options.

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

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

Learn about institutional subscriptions

References

  1. Kebebew E, Reiff E, Duh QY, Clark OH, McMillan A. Extent of disease at presentation and outcome for adrenocortical carcinoma: have we made progress? World J Surg. 2006;30:872–8.

    Article  PubMed  Google Scholar 

  2. Golden SH, Robinson KA, Saldanha I, Anton B, Ladenson PW. Clinical review: prevalence and incidence of endocrine and metabolic disorders in the United States: a comprehensive review. J Clin Endocrinol Metab. 2009;94:1853–78.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  3. Wajchenberg BL, Albergaria Pereira MA, Medonca BB, Latronico AC, Campos Carneiro P, Alves VA, et al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer. 2000;88:711–36.

    Article  CAS  PubMed  Google Scholar 

  4. Allolio BFM. Clinical review: adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab. 2006;91(6):2027–37.

    Article  CAS  PubMed  Google Scholar 

  5. Dackiw APB, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg. 2001;25:914–26.

    Article  CAS  PubMed  Google Scholar 

  6. Icard P, Goudet P, Charpenay C, Andreassian B, Carnaille B, Chapuis Y, et al. Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Surg. 2001;25:891–7.

    Article  CAS  PubMed  Google Scholar 

  7. Lughezzani G, Sun M, Perrotte P, Jeldres C, Alasker A, Isbarn H, et al. The European Network for the Study of Adrenal Tumors staging system is prognostically superior to the international union against cancer-staging system: a North American validation. Eur J Cancer. 2010;46:713–9.

    Article  PubMed  Google Scholar 

  8. Koschker A-C, Fassnacht M, Hahner S, Weismann D, Allolio B. Adrenocortical carcinoma – improving patient care by establishing new structures. Exp Clin Endocrinol Diabetes. 2006;114:45–51.

    Article  CAS  PubMed  Google Scholar 

  9. Hsing AW, Nam JM, Co Chien HT, McLaughlin JK, Fraumeni JF. Risk factors for adrenal cancer: an exploratory study. Int J Cancer. 1996;65:432–6.

    Article  CAS  PubMed  Google Scholar 

  10. Hisada M, Garber JE, Fung CY, Fraumeni JF, Li FP. Multiple primary cancers in families with Li-Fraumeni syndrome. J Natl Cancer Inst. 1998;90:606–11.

    Article  CAS  PubMed  Google Scholar 

  11. Fassnacht M, Libé R, Kroiss M, Allolio B. Adrenocortical carcinoma: a clinician’s update. Nat Rev Endocrinol. 2011;7:323–35.

    Article  CAS  PubMed  Google Scholar 

  12. McFarlane D. Cancer of the adrenal cortex; the natural history, pronosis and treatment in a study of fifty-five cases. Ann R Coll Surg Engl. 1958;23:155–86.

    Google Scholar 

  13. Sullivan M, Boileau MHC. Adrenal cortical carcinoma. J Urol. 1978;120:660–5.

    Article  CAS  PubMed  Google Scholar 

  14. DeLellis RA, Lloyd RV, Heitz PU, Eng C. World Health Organization classification of tumours. Pathology and genetics of tumours of endocrine organs. Lyon: IARC; 2004.

    Google Scholar 

  15. Fassnacht M, Johanssen S, Quinkler M, Bucsky P, Willenberg HS, Beuschlein F, et al. Limited prognostic value of the 2004 international union against cancer staging classification for adrenocortical carcinoma. Cancer. 2009;115:243–50.

    Article  PubMed  Google Scholar 

  16. Pommier RF, Brennan MF. An eleven-year experience with adrenocortical carcinoma. Surgery. 1992;112:963–70. Discussion 970–1.

    PubMed  CAS  Google Scholar 

  17. Stojadinovic A, Ghossein RA, Hoos A, Nissan A, Marshall D, Dudas M, et al. Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol. 2002;20:941–50.

    Article  CAS  PubMed  Google Scholar 

  18. Crucitti F, Bellantone R, Ferrante A, Boscherini M, Crucitti P, Carbone G, et al. The italian registry for adrenal cortical carcinoma: analysis of a multiinstitutional series of 129 patients. Surgery. 1996;119:161–70.

    Article  CAS  PubMed  Google Scholar 

  19. Linos D, Vassilopoulos P, Papadimitriou J, Tountas K. The surgical management of adrenal cortical carcinoma. Int Surg. 1986;71(2):104–6.

    PubMed  CAS  Google Scholar 

  20. Decker R, Kuehner M. Adrenocortical carcinoma. Am Surg. 1991;57(8):502–13.

    PubMed  CAS  Google Scholar 

  21. Jensen C. Recurrent or metastatic disease in select patients with adrenocortical carcinoma adrenocortical. Arch Surg. 1991;126:457–61.

    Article  CAS  PubMed  Google Scholar 

  22. Van Aalderen W, Van Seters AP, Backer ET, Chang PC, Van Krieken JHJM, Moolenaar AJ. A case of recurrent adrenocortical carcinoma, with observations on long-term o,p’-DDD therapy and complications. Neth J Med. 1992;41:161–70.

    PubMed  Google Scholar 

  23. Ahlman H, Jansson S, Wangberg B, Tisell L, Schersten T, Hansson G, et al. Adrenocortical carcinoma – diagnostic and therapeutical implications. Eur J Surg. 1993;159(3):149–58.

    PubMed  CAS  Google Scholar 

  24. Haak HR, Hermans J, van de Velde CJ, Lentjes EG, Goslings BM, Fleuren GJ, et al. Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Br J Cancer. 1994;69:947–51.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  25. Sakamoto K, Ariyoshi A, Okazaki M. Metastatic adrenocortical carcinoma treated by repeated resection: a case report of long-term survival over 18 years. Int J Urol. 1995;2:50–2.

    Article  CAS  PubMed  Google Scholar 

  26. Bellantone R, Ferrante A, Boscherini M, Lombardi CP, Crucitti P, Crucitti F, et al. Role of reoperation in recurrence of adrenal cortical carcinoma: results from 188 cases collected in the Italian National Registry for Adrenal Cortical Carcinoma. Surgery. 1997;122:1212–8.

    Article  CAS  PubMed  Google Scholar 

  27. Khorram-Manesh A, Ahlman H, Jansson S, Wängberg B, Nilsson O, Jakobsson CE, et al. Adrenocortical carcimona: surgery and mitotane for treatment and steroid profiles for follow-up. World J Surg. 1998;22:605–12.

    Article  CAS  PubMed  Google Scholar 

  28. Schulick RD, Brennan MF. Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol. 1999;6:719–26.

    Article  CAS  PubMed  Google Scholar 

  29. Seki M, Nomura K, Hirohara D, Kanazawa M, Sawada T, Takasaki K, et al. Changes in neoplastic cell features and sensitivity to mitotane during mitotane-induced remission in a patient with recurrent, metastatic adrenocortical carcinoma. Endocr Relat Cancer. 1999;6(4):529–33.

    Article  CAS  PubMed  Google Scholar 

  30. Langer P, Bartsch D, Moebius E, Rothmund M, Nies C. Adrenocortical carcinoma – our experience with 11 cases. Langenbeck’s Arch Surg. 2000;385:393–7.

    Article  CAS  Google Scholar 

  31. Ilias I, Alevizaki M, Philippou G, Anastasiou E, Souvatzoglou A. Sustained remission of metastatic adrenal carcinoma during long-term administration of low-dose mitotane. J Endocrinol Investig. 2001;24:532–5.

    Article  CAS  Google Scholar 

  32. Fujii Y, Kageyama Y, Kawakami S, Masuda H, Arisawa C, Akamatsu H, et al. Successful long-term disease-free survival following multimodal treatments in a patient with a repeatedly recurrent refractory adrenal cortical carcinoma. Int J Urol. 2003;10(8):445.

    Article  PubMed  Google Scholar 

  33. Tauchmanovà L, Colao A, Marzano LA, Sparano L, Camera L, Rossi A, et al. Andrenocortical carcinomas: twelve-year prospective experience. World J Surg. 2004;28:896–903.

    Article  PubMed  Google Scholar 

  34. Matsumoto K, Egawa S, Satoh T, Okuno N, Kaseda S, Baba S. Thoracoscopic transdiaphragmatic adrenalectomy for isolated locally recurrent adrenal carcinoma. Int J Urol. 2005;12:1055–7.

    Article  PubMed  Google Scholar 

  35. Palazzo FF, Sebag F, Sierra M, Ippolito G, Souteyrand P, Henry JF. Long-term outcome following laparoscopic adrenalectomy for large solid adrenal cortex tumors. World J Surg. 2006;30:893–8.

    Article  PubMed  Google Scholar 

  36. Schlamp A, Hallfeldt K, Mueller-Lisse U, Pfluger T, Reincke M. Recurrent adrenocortical carcinoma after laparoscopic resection. Nat Clin Pract Endocrinol Metab. 2007;3:191–5.

    Article  PubMed  Google Scholar 

  37. Tan CT, Meyer-Rochow GY, Sywak MS, Delbridge LW, Sidhu SB. Reoperative adrenal surgery: lessons learnt. ANZ J Surg. 2009;79:371–7.

    Article  PubMed  Google Scholar 

  38. Sabolch A, Feng M, Griffith K, Hammer G, Doherty G, Ben-Josef E. Adjuvant and definitive radiotherapy for adrenocortical carcinoma. Int J Radiat Oncol Biol Phys. 2011;80:1477–84.

    Article  PubMed  Google Scholar 

  39. Datrice NM, Langan RC, Ripley RT, Kemp CD, Steinberg SM, Wood BJ, et al. Operative management for recurrent and metastatic adrenocortical carcinoma. J Surg Oncol. 2012;105:709–13.

    Article  PubMed  Google Scholar 

  40. Dy BM, Wise KB, Richards ML, Young WF, Grant CS, Bible KC, et al. Operative intervention for recurrent adrenocortical cancer. Surgery. 2013;154:1292–9.

    Article  PubMed  Google Scholar 

  41. Erdogan I, Deutschbein T, Jurowich C, Kroiss M, Ronchi C, Quinkler M, et al. The role of surgery in the management of recurrent adrenocortical carcinoma. J Clin Endocrinol Metab. 2013;98:181–91.

    Article  CAS  PubMed  Google Scholar 

  42. Terzolo M, Angeli A, Fassnacht M, Daffara F, Tauchmanova L, Conton PA, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med. 2007;356:2372–80.

    Article  CAS  PubMed  Google Scholar 

  43. Saunders BD, Doherty GM. Laparoscopic adrenalectomy for malignant disease. Lancet Oncol. 2004;5:718–26.

    Article  PubMed  Google Scholar 

  44. Shen WT, Lim RC, Siperstein AE, Clark OH, Schecter WP, Hunt TK, et al. Laparoscopic vs open adrenalectomy for the treatment of primary hyperaldosteronism. Arch Surg. 1999;134:628–31.

    Article  CAS  PubMed  Google Scholar 

  45. Cobb WS, Kercher KW, Sing RF, Heniford BT. Laparoscopic adrenalectomy for malignancy. Am J Surg. 2005;189(4):405–11.

    Article  PubMed  Google Scholar 

  46. Hahner S, Fassnacht M. Mitotane for adrenocortical carcinoma treatment. Curr Opin Investig Drugs. 2005;6:386–94.

    PubMed  CAS  Google Scholar 

  47. Kopf D, Goretzki PE, Lehnert H. Clinical management of malignant adrenal tumors. J Cancer Res Clin Oncol. 2001;127:143–55.

    Article  CAS  PubMed  Google Scholar 

  48. Terzolo M, Pia A, Berruti A, Osella G, Alì A, Carbone V, et al. Low-dose monitored mitotane treatment achieves the therapeutic range with manageable side effects in patients with adrenocortical cancer. J Clin Endocrinol Metab. 2000;85:2234–8.

    PubMed  CAS  Google Scholar 

  49. Faggiano A, Leboulleux S, Young J, Schlumberger M, Baudin E. Rapidly progressing high o,p’DDD doses shorten the time required to reach the therapeutic threshold with an acceptable tolerance: preliminary results. Clin Endocrinol. 2006;64:110–3.

    Article  CAS  Google Scholar 

  50. Schteingart DE. Conventional and novel strategies in the treatment of adrenocortical cancer. Braz J Med Biol Res. 2000;33:1197–200.

    Article  CAS  PubMed  Google Scholar 

  51. Berruti A, Terzolo M, Sperone P, Pia A, Della Casa S, Gross DJ, et al. Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. Endocr Relat Cancer. 2005;12:657–66.

    Article  CAS  PubMed  Google Scholar 

  52. Khan TS, Imam H, Juhlin C, Skogseid B, Gröndal S, Tibblin S, et al. Streptozocin and o,p’DDD in the treatment of adrenocortical cancer patients: long-term survival in its adjuvant use. Ann Oncol. 2000;11:1281–7.

    Article  CAS  PubMed  Google Scholar 

  53. Fassnacht M, Terzolo M, Allolio B, Baudin E, Haak H, Berruti A, et al. Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med. 2012;366:2189–97.

    Article  CAS  PubMed  Google Scholar 

  54. Berruti A, Baudin E, Gelderblom H, Haak HR, Porpiglia F, Fassnacht M, et al. Adrenal cancer: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012;23(suppl 7):vii131–8.

    Article  PubMed  Google Scholar 

  55. Kornely E, Schlaghecke R. Complete remission of metastasized adrenocortical carcinoma under o,p’-DDD. Exp Clin Endocrinol. 1994;102:50–3.

    Article  CAS  PubMed  Google Scholar 

  56. Polat B, Fassnacht M, Pfreundner L, Guckenberger M, Bratengeier K, Johanssen S, et al. Radiotherapy in adrenocortical carcinoma. Cancer. 2009;115:2816–23.

    Article  PubMed  Google Scholar 

  57. Wood BJ, Abraham J, Hvizda JL, Alexander HR, Fojo T. Radiofrequency ablation of adrenal tumors and adrenocortical carcinoma metastases. Cancer. 2003;97:554–60.

    Article  PubMed  Google Scholar 

  58. Venkatesan AM, Locklin J, Dupuy DE, Wood BJ. Percutaneous ablation of adrenal Tumors. Tech Vasc Interv Radiol. 2010;13:89–99.

    Article  PubMed Central  PubMed  Google Scholar 

  59. Beland MD, Mayo-Smith WW. Ablation of adrenal neoplasms. Abdom Imaging. 2009;34:588–92.

    Article  PubMed  Google Scholar 

  60. Atwell TD, Wass CT, Charboneau JW, Callstrom MR, Farrell MA, Sengupta S. Malignant hypertension during cryoablation of an adrenal gland tumor. J Vasc Interv Radiol. 2006;17:573–5.

    Article  PubMed  Google Scholar 

  61. Xiao YY, Tian JL, Li JK, Yang L, Zhang JS. CT-guided percutaneous chemical ablation of adrenal neoplasms. Am J Roentgenol. 2008;190:105–10.

    Article  Google Scholar 

  62. Li SH, Huang CH, Ko SF, Chou FF, Huang SC. Extended survival in a patient with recurrent and metastatic adrenal cortical carcinoma by aggressive transarterial embolization – a case report. J Surg Oncol. 2005;90:101–5.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of General Surgery, Cleveland Clinic, Cleveland, OH, USA

    Zahraa Al-Hilli

  2. Department of Surgery, Mayo Clinic Hospital, Rochester, MN, USA

    Melanie L. Lyden

Authors
  1. Zahraa Al-Hilli
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Melanie L. Lyden
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Zahraa Al-Hilli .

Editor information

Editors and Affiliations

  1. Department of Surgery and MacLean Center for Clinical Medical Ethics, The University of Chicago, Chicago, IL, USA

    Peter Angelos

  2. Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA

    Raymon H. Grogan

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

Al-Hilli, Z., Lyden, M.L. (2018). Surgery Versus Nonsurgical Therapy for Recurrent Adrenocortical Carcinoma. 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_31

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-92860-9_31

  • 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

Search

Navigation