Management of Adrenocortical Carcinoma

Editor:
Kerrin G. Robinson Journal of the National Comprehensive Cancer Network

Search for other papers by Kerrin G. Robinson in
Current site
Google Scholar
PubMed
Close
 MA
Authors:
Jonathan R. Strosberg From the H. Lee Moffitt Cancer Center, Tampa, Florida, and University of Michigan School of Medicine, Ann Arbor, Michigan.

Search for other papers by Jonathan R. Strosberg in
Current site
Google Scholar
PubMed
Close
 MD
,
Gary D. Hammer From the H. Lee Moffitt Cancer Center, Tampa, Florida, and University of Michigan School of Medicine, Ann Arbor, Michigan.

Search for other papers by Gary D. Hammer in
Current site
Google Scholar
PubMed
Close
 MD
, and
Gerard M. Doherty From the H. Lee Moffitt Cancer Center, Tampa, Florida, and University of Michigan School of Medicine, Ann Arbor, Michigan.

Search for other papers by Gerard M. Doherty in
Current site
Google Scholar
PubMed
Close
 MD
Full access

Adrenocortical carcinomas (ACCs) are rare tumors that arise from the cortex of the adrenal gland with an incidence 1 to 2 per million. The rarity of this tumor translates into a paucity of experience in managing patients in most medical centers. Because clinical series are small and prospective evaluation of treatment strategies is limited, the current state of knowledge is strongly influenced by expert consensus opinion from a few medical centers specializing in ACCs. This article describes the basic diagnostic and prognostic issues in adrenal cancer management, and presents detailed rationales for therapeutic management.

Correspondence: Gerard M. Doherty, MD, University of Michigan, 2920 Taubman Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109. E-mail: gerardd@med.umich.edu

Disclosure: Jonathan R. Strosberg, MD, has disclosed no relevant financial relationships.

Disclosure: Gary D. Hammer, MD, has disclosed no relevant financial relationships.

Disclosure: Gerard M. Doherty, MD, has disclosed no relevant financial relationships.

Disclosure: Kerrin G. Robinson, MA, has disclosed no relevant financial relationships.

  • Collapse
  • Expand
  • 1

    Dackiw AP, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg 2001;25:914926.

  • 2

    Allolio B, Fassnacht M. Clinical review: adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 2006;91:20272037.

  • 3

    Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003;169:511.

  • 4

    Schteingart DE, Doherty GM, Gauger PG et al.. Management of patients with adrenal cancer: recommendations of an international consensus conference. Endocr Relat Cancer 2005;12:667680.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Crucitti F, Bellantone R, Ferrante A et al.. The Italian Registry for Adrenal Cortical Carcinoma: analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery 1996;119:161170.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Wooten MD, King DK. Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature. Cancer 1993;72:31453155.

  • 7

    Koch CA, Pacak K, Chrousos GP. The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab 2002;87:53675384.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Lynch HT, Radford B, Lynch JF. SBLA syndrome revisited. Oncology 1990;47:7579.

  • 9

    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;86:56585671.

  • 10

    Marx S, Spiegel AM, Skarulis MC et al.. Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med 1998;129:484494.

  • 11

    Soon PS, McDonald KL, Robinson BG, Sidhu SB. Molecular markers and the pathogenesis of adrenocortical cancer. Oncologist 2008;13:548561.

  • 12

    Reincke M, Karl M, Travis WH et al.. p53 mutations in human adrenocortical neoplasms: immunohistochemical and molecular studies. J Clin Endocrinol Metab 1994;78:790794.

  • 13

    Ohgaki H, Kleihues P, Heitz PU. p53 mutations in sporadic adrenocortical tumors. Int J Cancer 1993;54:408410.

  • 14

    Gicquel C, Bertagna X, Schneid H et al.. Rearrangements at the 11p15 locus and overexpression of insulin-like growth factor-II gene in sporadic adrenocortical tumors. J Clin Endocrinol Metab 1994;78:14441453.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Gicquel C, Raffin-Sanson ML, Gaston V et al.. Structural and functional abnormalities at 11p15 are associated with the malignant phenotype in sporadic adrenocortical tumors: study on a series of 82 tumors. J Clin Endocrinol Metab 1997;82:25592565.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16

    Barlaskar FM, Hammer GD. The molecular genetics of adrenocortical carcinoma. Rev Endocr Metab Disord 2007;8:343348.

  • 17

    Luton JP, Cerdas S, Billaud L et al.. Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 1990;322:11951201.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Bertagna C, Orth DN. Clinical and laboratory findings and results of therapy in 58 patients with adrenocortical tumors admitted to a single medical center (1951 to 1978). Am J Med 1981;71:855875.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Kasperlik-Zaluska AA, Migdalska BM, Zgliczynski S, Makowska AM. Adrenocortical carcinoma. A clinical study and treatment results of 52 patients. Cancer 1995;75:25872591.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    NIH state-of-the-science statement on management of the clinically inapparent adrenal mass (“incidentaloma”). NIH Consens State Sci Statements 2002;19:125.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21

    Korobkin M, Lombardi TJ, Aisen AM et al.. Characterization of adrenal masses with chemical shift and gadolinium-enhanced MR imaging. Radiology 1995;197:411418.

  • 22

    Yun M, Kim W, Alnafisi N et al.. 18 F-FDG PET in characterizing adrenal lesions detected on CT or MRI. J Nucl Med 2001;42:17951799.

  • 23

    Caoili EM, Korobkin M, Brown RK et al.. Differentiating adrenal adenomas from nonadenomas using (18)F-FDG PET/CT: quantitative and qualitative evaluation. Acad Radiol 2007;14:468475.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Al-Hawary MM, Francis IR, Korobkin M. Non-invasive evaluation of the incidentally detected indeterminate adrenal mass. Best Pract Res Clin Endocrinol Metab 2005;19:277292.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    Szolar DH, Korobkin M, Reittner P et al.. Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT [see comment]. Radiology 2005;234:479485.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26

    Hussain HK, Korobkin M. MR imaging of the adrenal glands. Magn Reson Imaging Clin N Am 2004;12:515544.

  • 27

    Israel GM, Korobkin M, Wang C et al.. Comparison of unenhanced CT and chemical shift MRI in evaluating lipid-rich adrenal adenomas. AJR Am J Roentgenol 2004;183:215219.

  • 28

    Becherer A, Vierhapper H, Potzi C et al.. FDG-PET in adrenocortical carcinoma. Cancer Biother Radiopharm 2001;16:289295.

  • 29

    Leboulleux S, Dromain C, Bonniaud G et al.. Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography. J Clin Endocrinol Metab 2006;91:920925.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Schultz CL, Haaga JR, Fletcher BD et al.. Magnetic resonance imaging of the adrenal glands: a comparison with computed tomography. AJR Am J Roentgenol 1984;143:12351240.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31

    Chang A, Glazer HS, Lee JK et al.. Adrenal gland: MR imaging. Radiology 1987;163:123128.

  • 32

    Weiss LM, Medeiros LJ, Vickery AL. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 1989;13:202206.

  • 33

    Aubert S, Wacrenier A, Leroy X et al.. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol 2002;26:16121619.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    DeLellis RA, Lloyd RV, Heitz PU, Eng C, eds. World Health Organization Classification of Tumours: Pathology & Genetics of Tumours of Endocrine Organs. Lyon: IARC Press; 2004.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35

    Macfarlane DA. Cancer of the adrenal cortex. Ann R Coll Surg 1958;23:155186.

  • 36

    Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 1984;8:163169.

  • 37

    Assie G, Antoni G, Tissier F et al.. Prognostic parameters of metastatic adrenocortical carcinoma. J Clin Endocrinol Metab 2007;92:148154.

  • 38

    Icard P, Goudet P, Charpenay C 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;25:891897.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Saunders BD, Doherty GM. Laparoscopic adrenalectomy for malignant disease. Lancet Oncol 2004;5:718726.

  • 40

    Dickstein G, Shechner C, Arad E et al.. Is there a role for low doses of mitotane (o,p’-DDD) as adjuvant therapy in adrenocortical carcinoma? J Clin Endocrinol Metab 1998;83:31003103.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    Khorram-Manesh A, Ahlman H, Jansson S et al.. Adrenocortical carcinoma: surgery and mitotane for treatment and steroid profiles for follow-up. World J Surg 1998;22:605611; discussion 611–612.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Vassilopoulou-Sellin R, Guinee VF, Klein MJ et al.. Impact of adjuvant mitotane on the clinical course of patients with adrenocortical cancer. Cancer 1993;71:31193123.

  • 43

    Terzolo M, Angeli A, Fassnacht M et al.. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med 2007;356:23722380.

  • 44

    Schteingart DE. Adjuvant mitotane therapy of adrenal cancer—use and controversy [see comment]. N Engl J Med 2007;356:24152418.

  • 45

    Haak HR, Hermans J, van de Velde CJ et al.. Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Br J Cancer 1994;69:947951.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 46

    Hutter AM Jr, Kayhoe DE. Adrenal cortical carcinoma. Results of treatment with o,p’DDD in 138 patients. Am J Med 1966;41:581592.

  • 47

    Magee BJ, Gattamaneni HR, Pearson D. Adrenal cortical carcinoma: survival after radiotherapy. Clin Radiol 1987;38:587588.

  • 48

    Percarpio B, Knowlton AH. Radiation therapy of adrenal cortical carcinoma. Acta Rad Ther Phys Biol 1976;15:288.

  • 49

    Fassnacht M, Hahner S, Polat B et al.. Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma [see comment]. J Clin Endocrinol Metab 2006;91:45014504.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 50

    Hahner S, Fassnacht M. Mitotane for adrenocortical carcinoma treatment. Curr Opin Investig Drugs 2005;6:386394.

  • 51

    Lubitz JA, Freeman L, Okun R. Mitotane use in inoperable adrenal cortical carcinoma. JAMA 1973;223:11091112.

  • 52

    Venkatesh S, Hickey RC, Sellin RV et al.. Adrenal cortical carcinoma. Cancer 1989;64:765769.

  • 53

    Berruti A, Terzolo M, Sperone P 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;12:657666.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 54

    Khan TS, Imam H, Juhlin C et al.. Streptozocin and o,p’DDD in the treatment of adrenocortical cancer patients: long-term survival in its adjuvant use. Ann Oncol 2000;11:12811287.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 55

    Koschker AC, Fassnacht M, Hahner S et al.. Adrenocortical carcinoma—improving patient care by establishing new structures. Exp Clin Endocrinol Diabetes 2006;114:4551.

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 1079 118 21
PDF Downloads 807 133 10
EPUB Downloads 0 0 0