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Tumours of the Adrenal Gland

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Abstract

Tumours of the adrenal gland are very common. They can be functional or non-functional, benign or malignant or arise in the adrenal cortex or medulla. The majority of adrenal tumours are benign non-functioning adenomas that are found incidentally on abdominal imaging for another cause (adrenal incidentaloma). Due to the risk of malignancy, incidentalomas over 6 cm should be removed while those under 4 cm may be followed with serial imaging. Benign functioning adrenal adenomas can secrete aldosterone, cortisol or androgens. After appropriate work-up, all functioning adrenal lesions should be surgically removed. 60 % of cases of adrenocortical carcinoma present with symptoms of hormone excess. After work-up, localized disease is treated with surgical resection and consideration of adjuvant mitotane. Pheochromocytomas may be benign or malignant. Essential components of perioperative management include alpha blockage followed by beta blockade.

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References

  1. Grumbach MM, Biller BM, Braunstein GD, et al. Management of the clinically inapparent adrenal mass (“incidentaloma”). Ann Intern Med. 2003;138(5):424–9.

    Article  PubMed  Google Scholar 

  2. Mansmann G, Lau J, Balk E, et al. The clinically inapparent adrenal mass: update in diagnosis and management. Endocr Rev. 2004;25(2):309–40.

    Article  PubMed  Google Scholar 

  3. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003;169(1):5–11.

    Article  PubMed  Google Scholar 

  4. Harari A, Inabnet 3rd WB. Malignant pheochromocytoma: a review. Am J Surg. 2011;201(5):700–8.

    Article  PubMed  Google Scholar 

  5. Funder JW, Carey RM, Fardella C, et al. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2008;93(9):3266–81.

    Article  CAS  PubMed  Google Scholar 

  6. Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008;93(5):1526–40.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Mattsson C, Young Jr WF. Primary aldosteronism: diagnostic and treatment strategies. Nat Clin Pract Nephrol. 2006;2(4):198–208. quiz, 1 p following 230.

    Article  CAS  PubMed  Google Scholar 

  8. Young WF, Stanson AW, Thompson GB, et al. Role for adrenal venous sampling in primary aldosteronism. Surgery. 2004;136(6):1227–35.

    Article  PubMed  Google Scholar 

  9. Koch CA, Pacak K, Chrousos GP. The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab. 2002;87(12):5367–84.

    Article  CAS  PubMed  Google Scholar 

  10. Hisada M, Garber JE, Fung CY, et al. Multiple primary cancers in families with Li-Fraumeni syndrome. J Natl Cancer Inst. 1998;90(8):606–11.

    Article  CAS  PubMed  Google Scholar 

  11. Brandi ML, Gagel RF, Angeli A, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86(12):5658–71.

    Article  CAS  PubMed  Google Scholar 

  12. Fassnacht M, Johanssen S, Quinkler M, et al. Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer. 2009;115(2):243–50.

    Article  PubMed  Google Scholar 

  13. Bilimoria KY, Shen WT, Elaraj D, et al. Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors. Cancer. 2008;113(11):3130–6.

    Article  PubMed  Google Scholar 

  14. Weiss LM, Medeiros LJ, Vickery Jr AL. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol. 1989;13(3):202–6.

    Article  CAS  PubMed  Google Scholar 

  15. Stojadinovic A, Ghossein RA, Hoos A, et al. Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol. 2002;20(4):941–50.

    Article  CAS  PubMed  Google Scholar 

  16. Morimoto R, Satoh F, Murakami O, et al. Immunohistochemistry of a proliferation marker Ki67/MIB1 in adrenocortical carcinomas: Ki67/MIB1 labeling index is a predictor for recurrence of adrenocortical carcinomas. Endocr J. 2008;55(1):49–55.

    Article  PubMed  Google Scholar 

  17. Abiven G, Coste J, Groussin L, et al. Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients. J Clin Endocrinol Metab. 2006;91(7):2650–5.

    Article  CAS  PubMed  Google Scholar 

  18. http://www.ensat.org/page-1317312. Accessed Oct 4 2014.

  19. Terzolo M, Angeli A, Fassnacht M, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med. 2007;356(23):2372–80.

    Article  CAS  PubMed  Google Scholar 

  20. Polat B, Fassnacht M, Pfreundner L, et al. Radiotherapy in adrenocortical carcinoma. Cancer. 2009;115(13):2816–23.

    Article  PubMed  Google Scholar 

  21. Neumann HP, Bausch B, McWhinney SR, et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002;346(19):1459–66.

    Article  CAS  PubMed  Google Scholar 

  22. Chen H, Sippel RS, O’Dorisio MS, et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010;39(6):775–83.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Gimenez-Roqueplo AP, Favier J, Rustin P, et al. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. Cancer Res. 2003;63(17):5615–21.

    CAS  PubMed  Google Scholar 

  24. Averbuch SD, Steakley CS, Young RC, et al. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med. 1988;109(4):267–73.

    Article  CAS  PubMed  Google Scholar 

  25. Rose B, Matthay KK, Price D, et al. High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer. 2003;98(2):239–48.

    Article  CAS  PubMed  Google Scholar 

  26. Angeli A, Osella G, Ali A, Terzolo M. Adrenal incidentaloma: an overview of clinical and epidemiological data from the National Italian Study Group. Horm Res. 1997;47(4-6):279–83.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Vanessa Palter M.D., Ph.D., F.R.C.S.C. .

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© 2016 University of Toronto General Surgery Oncology Program

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Palter, V., Devon, K., Hallet, J., Kulkarni, G.S., Law, C.H.L., Urbach, D.R. (2016). Tumours of the Adrenal Gland. In: Wright, F., Escallon, J., Cukier, M., Tsang, M., Hameed, U. (eds) Surgical Oncology Manual. Springer, Cham. https://doi.org/10.1007/978-3-319-26276-5_1

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  • DOI: https://doi.org/10.1007/978-3-319-26276-5_1

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