World Journal of Surgery

, Volume 30, Issue 5, pp 872–878

Extent of Disease at Presentation and Outcome for Adrenocortical Carcinoma: Have We Made Progress?

  • Electron Kebebew
  • Emily Reiff
  • Quan-Yang Duh
  • Orlo H. Clark
  • Alex McMillan



Adrenocortical carcinoma (ACC), a rare and aggressive malignancy, accounts for up to14% of adrenal incidentalomas. The only chance of cure for ACC is diagnosis at an early stage; therefore, a main indication for adrenalectomy in patients with adrenal incidentaloma has been the potential risk of ACC. Recent studies suggest that this has led to earlier stage of ACC at diagnosis, more curative operations, and better survival.


We analyzed data on ACC from The National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database. Four equal time quartiles (1973–1979, 1980–1986, 1987–1993, and 1994–2000) were compared for changes in demographics, pathology, treatment, and cause-specific mortality.


The average age was 51.2 years (range: 1–97), and 45.9% of patients were men. The average tumor size was 12 cm (range: 2–36 cm), and only 4.2% were ≤ 6 cm. Most (88%) patients had surgical resection of their tumor, and external beam radiotherapy was used in only 12% of patients. Between the time quartiles compared (as well as annually), there was no significant difference at presentation in age at diagnosis, sex, race/ethnicity, tumor size, tumor grade, the frequency of distant metastasis, and overall TNM stage. Low tumor grade, lower stage of ACC, later time quartile, and surgical resection were associated with a lower cause-specific mortality by univariate analysis (P ≤ 0.002) and by multivariate analysis (P ≤ 0.031).


Although adrenal incidentalomas have become a common indication for adrenalectomy, this has not resulted in patients with ACC being diagnosed earlier or treated at a lower stage of disease at the national level. The most important predictors of survival in these patients are tumor grade, tumor stage, and surgical resection.


  1. 1.
    Sidhu S, Sywak M, Robinson B, et al. Adrenocortical cancer: recent clinical and molecular advances. Curr Opin Oncol 2004;16:13–18CrossRefPubMedGoogle Scholar
  2. 2.
    Allolio B, Hahner S, Weismann D, et al. Management of adrenocortical carcinoma. Clin Endocrinol (Oxf) 2004;60:273–287CrossRefGoogle Scholar
  3. 3.
    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;25:891–897CrossRefPubMedGoogle Scholar
  4. 4.
    Kendrick ML, Lloyd R, Erickson L, et al. Adrenocortical carcinoma: surgical progress or status quo? Arch Surg 2001;136:543–549CrossRefPubMedGoogle Scholar
  5. 5.
    Mansmann G, Lau J, Balk E, et al. The clinically inapparent adrenal mass: update in diagnosis and management. Endocr Rev 2004;25:309–340CrossRefPubMedGoogle Scholar
  6. 6.
    Chen DY, Sosa RE, Scherr DS. Treatment of adrenocortical carcinoma: contemporary outcomes. Curr Urol Rep 2004;5:65–72PubMedGoogle Scholar
  7. 7.
    Sturgeon C, Kebebew E. Laparoscopic adrenalectomy for malignancy. Surg Clin North Am 2004;84:755–774CrossRefPubMedGoogle Scholar
  8. 8.
    Warren JL, Harlan LC. Can cancer registry data be used to study cancer treatment? Med Care 2003;41:1003–1005CrossRefPubMedGoogle Scholar
  9. 9.
    Reynolds T. Report examines association between cancer and socioeconomic status. J Natl Cancer Inst 2003;95:1431–1433PubMedGoogle Scholar
  10. 10.
    Kadan-Lottick NS, Ness KK, Bhatia S, Gurney JG, et al. Survival variability by race and ethnicity in childhood acute lymphoblastic leukemia. J Am Med Assoc 2003;290:2008–2014CrossRefGoogle Scholar
  11. 11.
    Gatta G, Ciccolallo L, Capocaccia R, et al. Differences in colorectal cancer survival between European and US populations: the importance of sub-site and morphology. Eur J Cancer 2003;39:2214–2222CrossRefPubMedGoogle Scholar
  12. 12.
  13. 13.
  14. 14.
    Tauchmanova L, Colao A, Marzano LA, et al. Andrenocortical carcinomas: twelve-year prospective experience. World J Surg 2004;28:896–903CrossRefPubMedGoogle Scholar
  15. 15.
    Wajchenberg BL, Albergaria Pereira MA, et al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer 2000;88:711–736CrossRefPubMedGoogle Scholar
  16. 16.
    Wooten MD, King DK. Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature. Cancer 1993;72:3145–3155PubMedGoogle Scholar
  17. 17.
    Stojadinovic A, Ghossein RA, Hoos A, et al. Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol 2002;20:941–950CrossRefPubMedGoogle Scholar
  18. 18.
    Schulick RD, Brennan MF. Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol 1999;6:719–726CrossRefPubMedGoogle Scholar
  19. 19.
    Tritos NA, Cushing GW, Heatley G, et al. Clinical features and prognostic factors associated with adrenocortical carcinoma: Lahey Clinic Medical Center experience. Am Surg 2000;66:73–79PubMedGoogle Scholar
  20. 20.
    Harrison LE, Gaudin PB, Brennan MF. Pathologic features of prognostic significance for adrenocortical carcinoma after curative resection. Arch Surg 1999;134:181–185CrossRefPubMedGoogle Scholar
  21. 21.
    Haak HR, Cornelisse CJ, Hermans J, et al. Nuclear DNA content and morphological characteristics in the prognosis of adrenocortical carcinoma. Br J Cancer 1993;68:151–155PubMedGoogle Scholar
  22. 22.
    de Fraipont F, El Atifi M, Cherradi N, et al. Gene expression profiling of human adrenocortical tumors using complementary deoxyribonucleic acid microarrays identifies several candidate genes as markers of malignancy. J Clin Endocrinol Metab 2005;90:1819–1829CrossRefPubMedGoogle Scholar

Copyright information

© Société Internationale de Chirurgie 2006

Authors and Affiliations

  • Electron Kebebew
    • 1
    • 2
  • Emily Reiff
    • 1
  • Quan-Yang Duh
    • 1
  • Orlo H. Clark
    • 1
    • 2
  • Alex McMillan
    • 2
  1. 1.Department of SurgeryUniversity of California, San FranciscoSan Francisco
  2. 2.UCSF Comprehensive Cancer CenterUCSF/Mount Zion Medical Center, University of California, San FranciscoSan Francisco

Personalised recommendations