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Aldosterone-Producing Adrenocortical Carcinoma

  • Anna Patalano
  • Maria V. Cicala
  • Franco Mantero
Chapter

Abstract

In 1955 Conn described the syndrome of primary hyperaldosteronism (PHA) [1], which has come to be known as “Conn’s Syndrome” [2]. PHA is a group of disorders in which aldosterone production is inappropriately high, relatively autonomous from the renin-angiotensin system, and nonsuppressible by sodium loading. Such inappropriate production of aldosterone causes cardiovascular damage, suppression of plasma renin, hypertension, sodium retention, and potassium excretion that if prolonged and severe may lead to hypokalemia. PHA is commonly caused by adenoma (35%), bilateral adrenal hyperplasia (60%), or (rarely) by aldosterone producing adrenocortical carcinoma (APAC) (<1%) (Table 27.1) [3]. The first APAC was reported in 1955 by Foye and Feichtmeir [4] shortly after Conn’s original report. Aldosterone hypersecretion in adrenocortical carcinoma (ACC) is rare with only 58 patients being reported in a recent review [5]. The reported numbers of APAC amongst ACCs vary significantly. In one large series of ACCs, only 2.5% had developed hyperaldosteronism [6]. In a single center analysis on ACCs, which were subjected to operative management at the Mayo Clnic, the portion of APACs was 11%. Conversely, it has been estimated that hyperaldosteronism is due to APAC in only 1% of patients with PHA [7].

Keywords

Primary Hyperaldosteronism Adrenocortical Carcinoma Adrenal Adenoma Adrenocortical Tumor Aldosterone Production 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Conn JW (1955) Primary aldosteronism. J Lab Clin Med 45:661–664PubMedGoogle Scholar
  2. 2.
    Hajjar RH et al (1975) Adrenal cortical carcinoma- a study of 32 cases. Cancer 35:549–554CrossRefPubMedGoogle Scholar
  3. 3.
    Funder JW et al (2008) Endocrine society. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab Sep 93(9):3266–3281CrossRefGoogle Scholar
  4. 4.
    Foye LV Jr, Feichtmeir TV (1955) Adrenal cortical carcinoma producing solely mineralocorticoid effect. Am J Med 19:966–975CrossRefPubMedGoogle Scholar
  5. 5.
    Seccia TM et al (2005) Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn’s syndrome with an ominous clinical course. Endocr Relat Cancer 12:149–159CrossRefPubMedGoogle Scholar
  6. 6.
    Ng L, Libertino JM (2003) Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 169:5–11CrossRefPubMedGoogle Scholar
  7. 7.
    Mattsson C, Young WF Jr (2006) Primary aldosteronism: diagnostic and treatment strategies. Nat Clin Pract Nephrol 2:198–208CrossRefPubMedGoogle Scholar
  8. 8.
    Messer CK et al (2007) Concomitant secretion of glucocorticoid, androgens, and mineralcorticoid by an adrenocortical carcinoma: case report and review of literature. Endocr Pract. Jul–Aug 13(4):408–412Google Scholar
  9. 9.
    Hogan TF et al (1980) A clinical and pathological study of adrenal carcinoma. Cancer 45:2880–2883CrossRefPubMedGoogle Scholar
  10. 10.
    Libè R et al (2007) Adrenocortical cancer: pathophysiology and clinical management. Endocr Relat Cancer 14(1):13–28CrossRefPubMedGoogle Scholar
  11. 11.
    Lipsett MB et al (1963) Clinical and pathophysiologic aspects of adrenocortical carcinoma. Am J Med 35:374–383CrossRefPubMedGoogle Scholar
  12. 12.
    Salassa RM et al (1975) Primary aldosteronism and malignant adrenocortical neoplasia. Trans Am Clin Clin Assoc 86:163–169Google Scholar
  13. 13.
    Levine DS et al (1984) Isolated production of aldosterone by a malignant adrenal carcinoma yale. J Biol Med 57(6):833–841Google Scholar
  14. 14.
    Alterman SL et al (1969) Primary adrenocortical carcinoma causing aldosteronism. Cancer 24:602–609CrossRefPubMedGoogle Scholar
  15. 15.
    Brooks RV et al (1957) Potassium deficiency of renal and adrenal origin. Am J Med 23:391–407CrossRefPubMedGoogle Scholar
  16. 16.
    Crane MG et al (1965) Primary aldosteronism due to adrenal carcinoma. Ann Intern Med 63:494–503PubMedGoogle Scholar
  17. 17.
    Feldman S, Ravera JJ (1961) Arterial hypertension caused by a primary malignant adrenocortical tumor. Thorax Dec 10:284–288Google Scholar
  18. 18.
    Knapton PJ (1965) Hypokalemic alkalosis in adrenal carcinoma. Lancet ii:346Google Scholar
  19. 19.
    Santander S et al (1965) Case of probable mineralcorticoid excess without hypercortisolism due to carcinoma of the adrenal cortex. J Clin Endocrinol 25:1429–1435CrossRefGoogle Scholar
  20. 20.
    Zimmerman B et al (1959) Physiologic and surgical problems in the management of primary aldosteronism. Ann Surg 150:653–664CrossRefGoogle Scholar
  21. 21.
    Abma EM et al (2008) Malignant aldosterone-producing adrenal tumor: reoccurrence with glucocorticoid excess without hyperaldosteronism. Neth J Med. Jun 66(6):252–255Google Scholar
  22. 22.
    Barzon L et al (1997) Adrenocortical carcinoma: experience in 45 patients. Oncology 54:490–496CrossRefPubMedGoogle Scholar
  23. 23.
    Hisamatsu H et al (2002) Adrenocortical carcinoma with primary aldosteronism associated with Cushing syndrome during recurrence. BJU Int 90:971–972CrossRefPubMedGoogle Scholar
  24. 24.
    Lack EE Adrenal cortical carcinoma. In: Lack EE (ed) Tumors of the adrenal gland and extradrenal paraganglia. Armed Forces Institute of Pathology, Washington, DC, pp 123–147Google Scholar
  25. 25.
    Ehrlich EN et al (1963) J Clin Endocrinol Metab 23:358CrossRefGoogle Scholar
  26. 26.
    Todesco S et al (1975) Primary Aldosteronism due to a malignant ovarian tumor. JCEM 41(5):809–819PubMedGoogle Scholar
  27. 27.
    Rossi GP et al (2000) A thoracic mass with hypertension and hypokalaemia. Lancet 356(9241):1570CrossRefPubMedGoogle Scholar
  28. 28.
    Flanagan MJ, McDonald JH (1967) Heterotopic adrenocortical adenoma producing primary aldosteronism. J Urol 98:133–139PubMedGoogle Scholar
  29. 29.
    Zubair M et al (2009) Transgenic expression of Ad4BP/SF-1 in fetal adrenal progenitor cells leads to ectopic adrenal formation. Mol Endocrinol 23:1657–1667CrossRefPubMedGoogle Scholar
  30. 30.
    Gicquel C, Le Bouc Y (1997) Molecular markers for malignancy in adrenocortical tumors. Horm Res 47(4–6):269–272CrossRefPubMedGoogle Scholar
  31. 31.
    Suzuki T et al (1992b) Discerning malignancy in human adrenocortical neoplasms: utility of DNA flow cytometry and immunohistochemistry. Mod Pathol May 5(3): 224–231Google Scholar
  32. 32.
    Ghnassia JP et al (1993) Adrenal cortical tumors. Prognostic evaluation of a series of 12 cases using anti-PCNA antibodies. Ann Pathol 13(5):312–316PubMedGoogle Scholar
  33. 33.
    Reincke M et al (1994) p53 mutations in human adrenocortical neoplasms: immunohistochemical and molecular studies. J Clin Endocrinol Metab Mar 78(3):790–794CrossRefGoogle Scholar
  34. 34.
    Sasano H et al (1995) Transcription factor adrenal 4 binding protein as a marker of adrenocortical malignancy. Hum Pathol Oct 26(10):1154–1156CrossRefGoogle Scholar
  35. 35.
    Mannelli M et al (2000) Telomerase activity is significantly enhanced in malignant adrenocortical tumors in comparison to benign adrenocortical adenomas. J Clin Endocrinol Metab Jan 85(1):468–470CrossRefGoogle Scholar
  36. 36.
    Greathouse DJ et al (1984) Pure primary hyperaldosteronism due to adrenal cortical carcinoma. Am J Med Jun 76(6):1132–1136CrossRefGoogle Scholar
  37. 37.
    Young WF Jr (1997) Pheochromocytoma and primary aldosteronism: diagnostic approaches. Endocrinol Metab Clin North Am Dec 26(4):801–827CrossRefGoogle Scholar
  38. 38.
    Deckers S et al (1999) Peritoneal carcinomatosis following laparoscopic resection of an adrenocortical tumor causing primary hyperaldosteronism. Horm Res 52(2):97–100CrossRefPubMedGoogle Scholar
  39. 39.
    Dixon AN, Bing RF (2001) Two cases of adrenocortical carcinoma presenting as Conn’s syndrome. J Hum Hypertens Jan 15(1):75–79CrossRefGoogle Scholar
  40. 40.
    Weingärtner K et al (1995) Isolated clinical syndrome of primary aldosteronism in a patient with adrenocortical carcinoma. Case report and review of the literature. Urol Int 55(4):232–235Google Scholar
  41. 41.
    Taylor W et al (1997) Adrenal carcinoma presenting as Conn’s syndrome. Aust N Z J Med Apr 27(2):201–202Google Scholar
  42. 42.
    Ali AE, Raphael SJ (2007) Funchonal Oncocytic adrenocortical carcinoma. Endocr Pathol Fall 18(3):187–189CrossRefGoogle Scholar
  43. 43.
    Schulick RD, Brennan MF (1999) Adrenocortical carcinoma. World J Urol Feb 17(1):26–34CrossRefGoogle Scholar
  44. 44.
    Cobb W et al (2005) Laparoscopic adrenalectomy for malignancy. Am J Surg 189:405–411CrossRefPubMedGoogle Scholar
  45. 45.
    Paton BL et al (2006) Outcomes of adrenal cortical carcinoma in the United States. Surgery 140:914–920CrossRefPubMedGoogle Scholar
  46. 46.
    Biglieri EG et al (1966) Postoperative studies of adrenal function in primary aldosteronism. J Clin Endocr 26:553–558CrossRefPubMedGoogle Scholar
  47. 47.
    Kendrick ML et al (2002) Aldosterone-secreting adrenocortical carcinomas are associated with unique operative risks and outcomes. Surgery 132:1008–1011CrossRefPubMedGoogle Scholar
  48. 48.
    Skogseid B et al (2008) Experience from an ongoing phase III study: FIRM-ACT. Abstract book of the 2nd annual international adrenal cancer symposium: clinical and basic science. March 14–15Google Scholar
  49. 49.
    Terzolo M et al (2007) Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med 356:2372–2380CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Anna Patalano
    • 1
  • Maria V. Cicala
    • 2
  • Franco Mantero
    • 2
  1. 1.Division of Endocrinology, Department of Medical and Surgical SciencesUniversity of PaduaPaduaItaly
  2. 2.Division of Endocrinology, Department of Medical and Surgical SciencesUniversity of PaduaPadovaItaly

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