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Clinical Presentation and Initial Diagnosis

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Adrenocortical Carcinoma

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Abstract

The initial clinical presentation of adrenocortical carcinoma (ACC) consists of signs and symptoms of hormone excess, local or regional manifestations of tumor growth, complications of metastatic disease, or more general manifestations of malignancy such as weight loss, fever, and malaise. Currently, an increasing number of patients is detected incidentally by modern imaging.

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References

  1. Abiven G et al (2006) 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 91(7):2650–2655

    Article  PubMed  CAS  Google Scholar 

  2. Crucitti F et al (1996) The Italian Registry for Adrenal Cortical Carcinoma: analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery 119(2):161–170

    Article  PubMed  CAS  Google Scholar 

  3. Dackiw AP et al (2001) Adrenal cortical carcinoma. World J Surg 25(7):914–926

    Article  PubMed  CAS  Google Scholar 

  4. Icard P et al (2001) Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Surg 25(7):891–897

    Article  PubMed  CAS  Google Scholar 

  5. Kendrick ML et al (2001) Adrenocortical carcinoma: surgical progress or status quo? Arch Surg 136(5):543–549

    Article  PubMed  CAS  Google Scholar 

  6. Koschker AC et al (2006) Adrenocortical carcinoma – improving patient care by establishing new structures. Exp Clin Endocrinol Diabetes 114(2):45–51

    Article  PubMed  CAS  Google Scholar 

  7. Schulick RD, Brennan MF (1999) Adrenocortical carcinoma. World J Urol 17(1):26–34

    Article  PubMed  CAS  Google Scholar 

  8. Wooten MD, King DK (1993) Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature. Cancer 72(11):3145–3155

    Article  PubMed  CAS  Google Scholar 

  9. Fassnacht M, Allolio B (2009) Clinical management of adrenocortical carcinoma. Best Pract Res Clin Endocrinol Metab 23(2):273–289

    Article  PubMed  CAS  Google Scholar 

  10. Didolkar MS et al (1981) Natural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients. Cancer 47(9):2153–2161

    Article  PubMed  CAS  Google Scholar 

  11. Favia G et al (2001) Adrenocortical carcinoma: is prognosis different in nonfunctioning tumors? Results of surgical treatment in 31 patients. World J Surg 25(6):735–738

    Article  PubMed  CAS  Google Scholar 

  12. Pommier RF, Brennan MF (1992) An eleven-year experience with adrenocortical carcinoma. Surgery 112(6):963–970; discussion 970–971

    PubMed  CAS  Google Scholar 

  13. Vassilopoulou-Sellin R, Schultz PN (2001) Adrenocortical carcinoma. Clinical outcome at the end of the 20th century. Cancer 92(5):1113–1121

    Article  PubMed  CAS  Google Scholar 

  14. Wajchenberg B et al (2000) Adrenocortical carcinoma: clinical and laboratory observations. Cancer 88(4):711–736

    Article  PubMed  CAS  Google Scholar 

  15. Newell-Price J et al (2006) Cushing’s syndrome. Lancet 367(9522):1605–1617

    Article  PubMed  CAS  Google Scholar 

  16. Stewart PM et al (1995) 11 beta-Hydroxysteroid dehydrogenase activity in Cushing’s syndrome: explaining the mineralocorticoid excess state of the ectopic adrenocorticotropin syndrome. J Clin Endocrinol Metab 80(12):3617–3620

    Article  PubMed  CAS  Google Scholar 

  17. Moreno S et al (2004) Profile and outcome of pure androgen-secreting adrenal tumors in women: experience of 21 cases. Surgery 136(6):1192–1198

    Article  PubMed  Google Scholar 

  18. Gabrilove J et al (1965) Feminizing adrenocortical tumors in the male: a review of 52 cases including a case report. Medicine 44: 37–39

    Article  PubMed  CAS  Google Scholar 

  19. Lanigan D et al (1993) A feminizing adrenocortical carcinoma presenting with gynaecomastia. Postgrad Med J 69(812):481–483

    Article  PubMed  CAS  Google Scholar 

  20. Allolio B et al (2004) Management of adrenocortical carcinoma. Clin Endocrinol (Oxf) 60:273–287

    Article  Google Scholar 

  21. 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(1):149–159

    Article  PubMed  Google Scholar 

  22. Barzon L et al (2005) Shift from Conn’s syndrome to Cushing’s syndrome in a recurrent adrenocortical carcinoma. Eur J Endocrinol 153(5):629–636

    Article  PubMed  CAS  Google Scholar 

  23. Hisamatsu H et al (2002) Adrenocortical carcinoma with primary aldosteronism associated with Cushing syndrome during recurrence. BJU Int 90(9):971–972

    Article  PubMed  CAS  Google Scholar 

  24. Hyodo T et al (1977) Adrenocortical carcinoma and hypoglycemia: evidence for production of nonsuppressible insulin-like activity by the tumor. J Clin Endocrinol Metab 44(6):1175–1184

    Article  PubMed  CAS  Google Scholar 

  25. Luton JP et al (1990) Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 322(17):1195–1201

    Article  PubMed  CAS  Google Scholar 

  26. Falchuk KR (1973) Inappropriate antidiuretic hormone-like syndrome associated with an adrenocortical carcinoma. Am J Med Sci 266(5):393–395

    Article  PubMed  CAS  Google Scholar 

  27. Yamanaka K et al (2000) A case of renin-producing adrenocortical cancer. Endocr J 47(2):119–125

    Article  PubMed  CAS  Google Scholar 

  28. Oka T et al (1996) Erythropoietin-producing adrenocortical carcinoma. Urol Int 56(4):246–249

    Article  PubMed  CAS  Google Scholar 

  29. Fragoso MC et al (2007) An inhibin B and estrogen-secreting adrenocortical carcinoma leading to selective FSH suppression. Horm Res 67(1):7–11

    Article  PubMed  CAS  Google Scholar 

  30. Pegoli W Jr. et al (1987) Ectopic calcitonin in adrenocortical carcinoma: a new tumor marker. J Pediatr Surg 22(12):1183–1184

    Article  PubMed  Google Scholar 

  31. Bussani R et al (2003) Chance diagnosis of low stage non-metastasized adrenal cortical carcinoma in a young woman with retroperitoneal hemorrhage. Pathol Res Pract 199(11):761–763

    Article  PubMed  Google Scholar 

  32. Suyama K et al (2007) Spontaneous rupture of adrenocortical carcinoma. Am J Surg 194(1):77–78

    Article  PubMed  Google Scholar 

  33. Chesson JP, Theodorescu D (2002) Adrenal tumor with caval extension – case report and review of the literature. Scand J Urol Nephrol 36(1):71–73

    Article  PubMed  Google Scholar 

  34. Hisham AN et al (2003) Large adrenocortical carcinoma extending into the inferior vena cava and right atrium. Asian J Surg 26(1):40–42

    Article  PubMed  CAS  Google Scholar 

  35. Radecka E et al (2003) An unusual case of tumor thrombus in the inferior vena cava. A case report. Acta Radiol 44(2):160–161

    Article  PubMed  CAS  Google Scholar 

  36. Yeh MW et al (2006) Virilizing adrenocortical carcinoma with cavoatrial extension. Am J Surg 192(2):209–210

    Article  PubMed  Google Scholar 

  37. Fassnacht M et al (2009) Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer 115(2):243–250

    Article  PubMed  Google Scholar 

  38. Bakthavathsalam G et al (2008) Nonfunctioning adrenocorticalcarcinoma. Int Surg 93(2):81–87

    PubMed  CAS  Google Scholar 

  39. Brand TC et al (2001) Adrenal cortical carcinoma presenting as right varicocele. J Urol 165(2):503

    Article  PubMed  CAS  Google Scholar 

  40. Wright CB et al (2008) Adrenocortical tumor with left renal vein, vena cava and intrahepatic venous extension. J Cardiovasc Surg (Torino) 49(1):79–81

    CAS  Google Scholar 

  41. Carbonnel F et al (1988) Acute Budd-Chiari syndrome as first manifestation of adrenocortical carcinoma. J Clin Gastroenterol 10(4):441–444

    Article  PubMed  CAS  Google Scholar 

  42. Schteingart DE et al (2001) Overexpression of CXC chemokines by an adrenocortical carcinoma: a novel clinical syndrome. J Clin Endocrinol Metab 86(8):3968–3974

    Article  PubMed  CAS  Google Scholar 

  43. Durusu M et al (2002) Adrenal cortical carcinoma presenting initially with radius metastasis. Clin Oncol (R Coll Radiol) 14(1):83–84

    Google Scholar 

  44. Solans R et al (2001) [Bone metastases as presentation of adrenocortical carcinoma]. Med Clin (Barc) 116(2):76–77

    CAS  Google Scholar 

  45. Macfarlane DA (1958) Cancer of the adrenal cortex: the natural history, prognosis and treatment in a study of fifty-five cases. Ann R C Surg Engl 23(3):155–186

    CAS  Google Scholar 

  46. Fassnacht M et al (2004) Adrenal tumors: how to establish malignancy? J Endocrinol Invest 27(4):387–399

    PubMed  CAS  Google Scholar 

  47. Elamin MB et al (2008) Accuracy of Diagnostic Tests for Cushing’s Syndrome: a Systematic Review and Metaanalyses. J Clin Endocrinol Metab 93(5):1553–1562

    Article  PubMed  CAS  Google Scholar 

  48. Nieman LK et al (2008) The Diagnosis of Cushing’s Syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 93(5):1526–1540

    Article  PubMed  CAS  Google Scholar 

  49. Boyle JG et al (2007) Comparison of diagnostic accuracy of urinary free metanephrines, vanillyl mandelic acid, and catecholamines and plasma catecholamines for diagnosis of pheochromocytoma. J Clin Endocrinol Metab 92(12):4602–4608

    Article  PubMed  CAS  Google Scholar 

  50. Eisenhofer G (2003) Editorial: biochemical diagnosis of pheochromocytoma – is it time to switch to plasma-free metanephrines? J Clin Endocrinol Metab 88(2):550–552

    Article  PubMed  CAS  Google Scholar 

  51. Pacak K et al (2007) Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab 3(2):92–102

    Article  PubMed  Google Scholar 

  52. Perry CG et al (2007) The diagnostic efficacy of urinary fractionated metanephrines measured by tandem mass spectrometry in detection of pheochromocytoma. Clin Endocrinol (Oxf) 66(5):703–708

    Article  CAS  Google Scholar 

  53. Unger N et al (2006) Diagnostic value of various biochemical parameters for the diagnosis of pheochromocytoma in patients with adrenal mass. Eur J Endocrinol 154(3):409–417

    Article  PubMed  CAS  Google Scholar 

  54. Vaclavik J et al (2007) Free plasma metanephrines as a screening test for pheochromocytoma in low-risk patients. J Hypertens 25(7):1427–1431

    Article  PubMed  CAS  Google Scholar 

  55. Arlt W et al (2008) Steroid profiling in the diagnosis and monitoring of adrenocortical cancer – results of the EURINE ACC Study of the European Network For The Study Of Adrenal Tumors (ENS@T). Abstracts of the 90th annual meeting of the Endocrine Society (San Francisco): OR40-2

    Google Scholar 

  56. Johanssen S, et al (2010) Need for improvement in the clinical management of patients with adrenocortical carcinoma in Germany. Dt Aerzteblatt in press

    Google Scholar 

  57. Allolio B, Fassnacht M (2006) Clinical review: adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 91(6):2027–2037

    Article  CAS  Google Scholar 

  58. Friedrich-Rust M et al (2008) Contrast-enhanced sonography of adrenal masses: differentiation of adenomas and nonadenomatous lesions. AJR Am J Roentgenol 191(6):1852–1860

    Article  PubMed  Google Scholar 

  59. Bauditz J et al (2008) Improved detection of hepatic metastases of adrenocortical cancer by contrast-enhanced ultrasound. Oncol Rep 19(5):1135–1139

    PubMed  Google Scholar 

  60. Boland GW et al (1998) Characterization of adrenal masses using unenhanced CT: an analysis of the CT literature. AJR Am J Roentgenol 171(1):201–204

    PubMed  CAS  Google Scholar 

  61. Caoili EM et al (2002) Adrenal masses: characterization with combined unenhanced and delayed enhanced CT. Radiology 222(3):629–633

    Article  PubMed  Google Scholar 

  62. Hamrahian AH et al (2005) Clinical utility of noncontrast computed tomography attenuation value (Hounsfield units) to differentiate adrenal adenomas/hyperplasias from nonadenomas: cleveland Clinic experience. J Clin Endocrinol Metab 90(2):871–877

    Article  PubMed  CAS  Google Scholar 

  63. Heinz-Peer G et al (2007) Imaging of adrenal masses. Curr Opin Urol 17(1):32–38

    Article  PubMed  Google Scholar 

  64. Ilias I et al (2007) The optimal imaging of adrenal tumours: a comparison of different methods. Endocr Relat Cancer 14(3):587–599

    Article  PubMed  Google Scholar 

  65. Szolar DH et al (2005) Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. Radiology 234(2):479–485

    Article  PubMed  Google Scholar 

  66. Park BK et al (2007) Comparison of delayed enhanced CT and chemical shift MR for evaluating hyperattenuating incidental adrenal masses. Radiology 243(3):760–765

    Article  PubMed  Google Scholar 

  67. Honigschnabl S et al (2002) How accurate is MR imaging in characterisation of adrenal masses: update of a long-term study. Eur J Radiol 41(2):113–122

    Article  PubMed  Google Scholar 

  68. Al-Hawary MM et al (2005) Non-invasive evaluation of the incidentally detected indeterminate adrenal mass. Best Pract Res Clin Endocrinol Metab 19(2):277–292

    Article  PubMed  Google Scholar 

  69. Faria JF et al (2007) Adrenal masses: characterization with in vivo proton MR spectroscopy – initial experience. Radiology 245(3):788–797

    Article  PubMed  Google Scholar 

  70. Groussin L et al (2009) 18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients. J Clin Endocrinol Metab 94(5):1713–1722

    Article  PubMed  CAS  Google Scholar 

  71. Boland GW et al (2009) PET/CT for the characterization of adrenal masses in patients with cancer: qualitative versus quantitative accuracy in 150 consecutive patients. AJR Am J Roentgenol 192(4):956–962

    Article  PubMed  Google Scholar 

  72. Okada M et al (2009) Adrenal masses: the value of additional fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) in differentiating between benign and malignant lesions. Ann Nucl Med 23(4):349–354

    Article  PubMed  Google Scholar 

  73. Leboulleux S et al (2006) Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography. J Clin Endocrinol Metab 91(3):920–925

    Article  PubMed  CAS  Google Scholar 

  74. Mackie GC et al (2006) Use of [18F]fluorodeoxyglucose positron emission tomography in evaluating locally recurrent and metastatic adrenocortical carcinoma. J Clin Endocrinol Metab 91(7):2665–271

    Article  PubMed  CAS  Google Scholar 

  75. DeLellis RA et al (2004) World Health Organization Classification of Tumours. Pathology and genetics of tumours of endocrine organs (IARC), Lyon, France, pp 136

    Google Scholar 

  76. Sullivan M et al (1978) Adrenal cortical carcinoma. J Urol 120(6):660–665

    PubMed  CAS  Google Scholar 

  77. Quayle FJ et al (2007) Needle biopsy of incidentally discovered adrenal masses is rarely informative and potentially hazardous. Surgery 142(4):497–502; discussion 502–504

    Article  PubMed  Google Scholar 

  78. Casola G et al (1986) Unsuspected pheochromocytoma: risk of blood-pressure alterations during percutaneous adrenal biopsy. Radiology 159(3):733–735

    PubMed  CAS  Google Scholar 

  79. Kloos RT et al (1995) Incidentally discovered adrenal masses. Endocr Rev 16(4):460–484

    PubMed  CAS  Google Scholar 

  80. Lumachi F et al (2001) Fine-needle aspiration cytology of adrenal masses in noncancer patients: clinicoradiologic and histologic correlations in functioning and nonfunctioning tumors. Cancer 93(5):323–329

    Article  PubMed  CAS  Google Scholar 

  81. McCorkell SJ, Niles NL (1985) Fine-needle aspiration of catecholamine-producing adrenal masses: a possibly fatal mistake. AJR Am J Roentgenol 145(1):113–114

    PubMed  CAS  Google Scholar 

  82. Yankaskas B et al (1986) Delayed complications from fine-needle biopsies of solid masses of the abdomen. Invest Radiol 21(4):325–328

    Article  PubMed  CAS  Google Scholar 

  83. Fassina AS et al (2000) Fine needle aspiration cytology (FNAC) of adrenal masses. Cytopathology 11(5):302–311

    Article  PubMed  CAS  Google Scholar 

  84. Habscheid W et al (1990) [Puncture track metastasis after ultrasound-guided fine-needle puncture biopsy. A rare complication?]. Dtsch Med Wochenschr 115(6):212–215

    Article  PubMed  CAS  Google Scholar 

  85. Mody MK et al (1995) Percutaneous CT-guided biopsy of adrenal masses: immediate and delayed complications. J Comput Assist Tomogr 19(3):434–439

    Article  PubMed  CAS  Google Scholar 

  86. de Agustin P, Lopez-Rios F et al (1999) Fine-needle aspiration biopsy of the adrenal glands: a ten-year experience. Diagn Cytopathol 21(2):92–97

    Article  PubMed  Google Scholar 

  87. Katz RL et al (1984) Fine needle aspiration cytology of the adrenal gland. Acta Cytol 28(3):269–282

    PubMed  CAS  Google Scholar 

  88. Silverman SG et al (1993) Predictive value of image-guided adrenal biopsy: analysis of results of 101 biopsies. Radiology 187(3):715–718

    PubMed  CAS  Google Scholar 

  89. Wadih G et al (1992) Fine-needle aspiration cytology of the adrenal gland. Fifty biopsies in 48 patients. Arch Pathol Lab Med 116(8):841–846

    PubMed  CAS  Google Scholar 

  90. Welch TJ et al (1994) Percutaneous adrenal biopsy: review of a 10-year experience. Radiology 193(2):341–344

    PubMed  CAS  Google Scholar 

  91. Wu HH et al (1998) Fine needle aspiration cytology of benign adrenal cortical nodules. A comparison of cytologic findings with those of primary and metastatic adrenal malignancies. Acta Cytol 42(6):1352–1358

    PubMed  CAS  Google Scholar 

  92. Ren R et al (2006) Fine-needle aspiration of adrenal cortical carcinoma: cytologic spectrum and diagnostic challenges. Am J Clin Pathol 126(3):389–398

    Article  PubMed  Google Scholar 

  93. Lumachi F et al (2003) CT-scan, MRI and image-guided FNA cytology of incidental adrenal masses. Eur J Surg Oncol 29(8):689–692

    Article  PubMed  CAS  Google Scholar 

  94. Saeger W et al (2003) High diagnostic accuracy of adrenal core biopsy: results of the German and Austrian adrenal network multicenter trial in 220 consecutive patients. Hum Pathol 34(2):180–186

    Article  PubMed  CAS  Google Scholar 

  95. Jhala NC et al (2004) Endoscopic ultrasound-guided fine-needle aspiration biopsy of the adrenal glands: analysis of 24 patients. Cancer 102(5):308–314

    Article  PubMed  Google Scholar 

  96. Haseganu LE, Diehl DL (2009) Left adrenal gland hemorrhage as a complication of EUS-FNA. Gastrointest Endosc 69(6):e51–52

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Internal Medicine I, Endocrine and Diabetes Unit, University of Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany

    Bruno Allolio & Martin Fassnacht

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  1. Bruno Allolio
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  2. Martin Fassnacht
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Correspondence to Bruno Allolio .

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Editors and Affiliations

  1. Zina Pitcher Place 109, Ann Arbor, 48109-2200, Michigan, USA

    Gary D. Hammer

  2. Dept. Internal Medicine, Div. Pulmonary & Critical Care Medicine, University of Michigan Health System, Zina Pitcher Place 109, Ann Arbor, 48109-2200, Michigan, USA

    Tobias Else

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Allolio, B., Fassnacht, M. (2009). Clinical Presentation and Initial Diagnosis. In: Hammer, G., Else, T. (eds) Adrenocortical Carcinoma. Springer, New York, NY. https://doi.org/10.1007/978-0-387-77236-3_4

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