Adrenal Carcinoma Follow-up

  • Author: Bagi RP Jana, MD; Chief Editor: Jules E Harris, MD   more...
 
Updated: Jan 6, 2012
 

Complications

  • Potential complications that may be associated with AC can be subclassified as follows:
    • Local tumor invasion, including the potential for tumor thrombus formation, which can embolize similar to renal cell carcinoma
    • Hormone excess syndromes (eg, Cushing syndrome, hyperaldosteronism, hirsutism, virilization, and hypertension)
    • Paraneoplastic syndromes (eg, cachexia)
    • Local pain in patients with bone metastases
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Prognosis

  • Detection of tumors at early clinical stage is crucial for curative resection.
  • Total resection offers the only prospect for cure.
  • A majority of cases of AC are metastatic at the time of diagnosis. The most common sites of spread are the local periadrenal tissue, lymph nodes, lungs, liver, and bone.
  • Patients with functional AC may have a better prognosis because they present earlier, unlike patients with nonfunctional variants, who invariably present when the tumors are very large or are associated with distant metastasis.
    • Estimates of the overall 5-year survival rate are approximately 20-35%.
    • For cases where total surgical resection is achieved, this rate is estimated to be approximately 32-47%.
    • In those cases where total surgical extirpation has not been possible, the 5-year survival rates are 10-30%.
    • Even after apparently complete surgical resection, local or distant relapse occurs in nearly 80% of cases.
  • Estrogen receptor (ER) negative status confers a worse prognosis in adrenocortical carcinoma. In a study of 17 patients, Shen et al found that 1- and 5-year survival rates were 86% and 60%, respectively, for patients with ER-positive tumors, versus 38% and 0% for those with ER-negative tumors (P< 0.05).[18]
  • The presence of distant metastasis generally is a sign of an especially poor outcome. Estimates suggest that as many as 50% of such patients are dead within 12 months of detecting the metastatic deposits, regardless of treatment.
    • The most important predictive clinical parameters of prognosis are disease stage at diagnosis, completeness of resection at surgery, and presence or absence of metastasis at the time of diagnosis.
    • Recent follow-up data from large centers, such as the MD Anderson Cancer Center, Memorial Sloan-Kettering Cancer Center, and the French association of Endocrine Surgery series from Europe, suggest a temporal improvement in clinical survival of patients with AC in more recent years since the late 1980s and early 1990s.
  • Although still somewhat controversial, some suggest that children with AC have a better prognosis than adults. Favorable clinical outcome has been reported in 70% or more of pediatric cases.[19]
  • Even for patients with curative surgery, life-long follow-up is mandatory because documented cases exist of AC recurrence more than 10 years after presumed curative surgery.
  • Recurrent or relapsing AC is usually a bad omen. Although symptoms of hormonal excess can often be medically managed in this setting, cure is virtually unknown, and finding metastatic disease gives a particularly poor prognosis. Most of these patients die within 1 year. The prognosis is better in children, in whom some cases of long-term survival have been described.
  • The prognosis for cases of AC occurring in pregnancy is equally grim; however, the fetal prognosis in these cases remains excellent.
  • Patients who show no response to mitotane or who relapse are probably best served by a referral a major cancer center, where they can be enrolled in one of several ongoing combination chemotherapeutic/radiation and/or surgical resection protocols. AC is too uncommon for most tertiary hospitals to have enough expertise to manage these patients adequately.
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Contributor Information and Disclosures
Author

Bagi RP Jana, MD  Assistant Professor, University of Texas Medical Branch, Galveston, TX

Bagi RP Jana, MD is a member of the following medical societies: American Cancer Society, American Medical Association, American Society of Clinical Oncology, and Southwest Oncology Group

Disclosure: Nothing to disclose.

Coauthor(s)

Kush Sachdeva, MD  Southern Oncology and Hematology Associates, South Jersey Healthcare, Fox Chase Cancer Center Partner

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Perry, MD, MS, MACP  Nellie B Smith Chair of Oncology Emeritus, Director, Division of Hematology and Medical Oncology, Deputy Director, Ellis Fischel Cancer Center, University of Missouri-Columbia School of Medicine

Michael Perry, MD, MS, MACP is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Clinical Oncology, American Society of Hematology, International Association for the Study of Lung Cancer, and Missouri State Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Benjamin Movsas, MD  Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center

Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD  Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine; Consulting Staff, Arizona Cancer Center

Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research

Disclosure: GlobeImmune Salary Consulting

Additional Contributors

Gabriel I Uwaifo, MBBS, and Antonio Tito Fojo, MD, are gratefully acknowledged for the contributions made to this article.

References

  1. Angeli A, Osella G, Alì 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. [Medline].

  2. Villelli NW, Jayanti MK, Zynger DL. Use and Usefulness of Adrenal Core Biopsies Without FNA or On-site Evaluation of Adequacy: A Study of 204 Cases for a 12-Year Period. Am J Clin Pathol. Jan 2012;137(1):124-31. [Medline].

  3. Lau SK, Weiss LM. The Weiss system for evaluating adrenocortical neoplasms: 25 years later. Hum Pathol. Jun 2009;40(6):757-68. [Medline].

  4. Soon PS, Gill AJ, Benn DE, Clarkson A, Robinson BG, McDonald KL, et al. Microarray gene expression and immunohistochemistry analyses of adrenocortical tumors identify IGF2 and Ki-67 as useful in differentiating carcinomas from adenomas. Endocr Relat Cancer. Jun 2009;16(2):573-83. [Medline].

  5. International Union Against Cancer. TNM Classification of MalignantTumours - 7th edition. Available at http://blogs.globalink.org/uicc/templates/uicc/pdf/tnm/tnm_7th_edition_summary_091016.pdf. Accessed December 1, 2009.

  6. Fassnacht M, Johanssen S, Quinkler M, Bucsky P, Willenberg HS, Beuschlein F, et al. Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer. Jan 15 2009;115(2):243-50. [Medline].

  7. Baudin E, Leboulleux S, Al Ghuzlan A, Chougnet C, Young J, Deandreis D, et al. Therapeutic management of advanced adrenocortical carcinoma: what do we know in 2011?. Horm Cancer. Dec 2011;2(6):363-71. [Medline].

  8. Terzolo M, Angeli A, Fassnacht M, Daffara F, Tauchmanova L, Conton PA, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med. Jun 7 2007;356(23):2372-80. [Medline].

  9. Grubbs EG, Callender GG, Xing Y, Perrier ND, Evans DB, Phan AT, et al. Recurrence of Adrenal Cortical Carcinoma Following Resection: Surgery Alone Can Achieve Results Equal to Surgery Plus Mitotane. Ann Surg Oncol. Oct 23 2009;[Medline].

  10. Ronchi CL, Sbiera S, Kraus L, Wortmann S, Johanssen S, Adam P, et al. Expression of excision repair cross complementing group 1 and prognosis in adrenocortical carcinoma patients treated with platinum-based chemotherapy. Endocr Relat Cancer. Sep 2009;16(3):907-18. [Medline].

  11. Fassnacht M, Allolio B. Clinical management of adrenocortical carcinoma. Best Pract Res Clin Endocrinol Metab. Apr 2009;23(2):273-89. [Medline].

  12. Polat B, Fassnacht M, Pfreundner L, Guckenberger M, Bratengeier K, Johanssen S, et al. Radiotherapy in adrenocortical carcinoma. Cancer. Jul 1 2009;115(13):2816-23. [Medline].

  13. Datrice NM, Langan RC, Ripley RT, Kemp CD, Steinberg SM, Wood BJ, et al. Operative management for recurrent and metastatic adrenocortical carcinoma. J Surg Oncol. Dec 20 2011;[Medline].

  14. Kazaryan AM, Marangos IP, Rosseland AR, Røsok BI, Villanger O, Pinjo E, et al. Laparoscopic adrenalectomy: Norwegian single-center experience of 242 procedures. J Laparoendosc Adv Surg Tech A. Apr 2009;19(2):181-9. [Medline].

  15. Zografos GN, Vasiliadis G, Farfaras AN, Aggeli C, Digalakis M. Laparoscopic surgery for malignant adrenal tumors. JSLS. Apr-Jun 2009;13(2):196-202. [Medline].

  16. Kulis T, Knezevic N, Pekez M, Kastelan D, Grkovic M, Kastelan Z. Laparoscopic Adrenalectomy: Lessons Learned from 306 Cases. J Laparoendosc Adv Surg Tech A. Dec 13 2011;[Medline].

  17. Kemp CD, Ripley RT, Mathur A, et al. Pulmonary resection for metastatic adrenocortical carcinoma: the National Cancer Institute experience. Ann Thorac Surg. Oct 2011;92(4):1195-200. [Medline].

  18. Shen XC, Gu CX, Qiu YQ, Du CJ, Fu YB, Wu JJ. Estrogen receptor expression in adrenocortical carcinoma. J Zhejiang Univ Sci B. Jan 2009;10(1):1-6. [Medline].

  19. Dehner LP, Hill DA. Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?. Pediatr Dev Pathol. Jul-Aug 2009;12(4):284-91. [Medline].

  20. Abramson SJ. Adrenal neoplasms in children. Radiol Clin North Am. Nov 1997;35(6):1415-53. [Medline].

  21. Agrons GA, Lonergan GJ, Dickey GE, Perez-Monte JE. Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics. Jul-Aug 1999;19(4):989-1008. [Medline].

  22. Al-Fiar FZ, Pantalony D, Shepherd F. Primary bilateral adrenal lymphoma. Leuk Lymphoma. Nov 1997;27(5-6):543-9. [Medline].

  23. Bornstein SR, Stratakis CA, Chrousos GP. Adrenocortical tumors: recent advances in basic concepts and clinical management. Ann Intern Med. May 4 1999;130(9):759-71. [Medline].

  24. Boscaro M, Fallo F, Barzon L, et al. Adrenocortical carcinoma: epidemiology and natural history. Minerva Endocrinol. Mar 1995;20(1):89-94. [Medline].

  25. Carney JA. Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin Proc. Jun 1999;74(6):543-52. [Medline].

  26. Coonrod DV, Rizkallah TH. Virilizing adrenal carcinoma in a woman of reproductive age: a case presentation and literature review. Am J Obstet Gynecol. Jun 1995;172(6):1912-4; discussion 1914-5. [Medline].

  27. De Leon DD, Lange BJ, Walterhouse D, Moshang T. Long-term (15 years) outcome in an infant with metastatic adrenocortical carcinoma. J Clin Endocrinol Metab. Oct 2002;87(10):4452-6.

  28. Demeure MJ, Somberg LB. Functioning and nonfunctioning adrenocortical carcinoma: clinical presentation and therapeutic strategies. Surg Oncol Clin N Am. Oct 1998;7(4):791-805. [Medline].

  29. Favia G, Lumachi F, Carraro P, D'Amico DF. Adrenocortical carcinoma. Our experience. Minerva Endocrinol. Mar 1995;20(1):95-9. [Medline].

  30. Fokaefs ED, Melekos MD, Melachrinou MP, et al. Nonfunctional adrenal cortical carcinoma. Report of a case and review of the literature. Int Urol Nephrol. 1996;28(2):145-52. [Medline].

  31. Freeman DA. Adrenal carcinoma. In: Bardin CW, ed. Current therapy in endocrinology and metabolism. 6th ed. St. Louis, Mo: Mosby Yearbook; 1997:. 173-5.

  32. Gicquel C, Baudin E, Lebouc Y, Schlumberger M. Adrenocortical carcinoma. Ann Oncol. May 1997;8(5):423-7. [Medline].

  33. Gicquel C, Bertagna X, Gaston V, et al. Molecular markers and long-term recurrences in a large cohort of patients with sporadic adrenocortical tumors. Cancer Res. Sep 15 2001;61(18):6762-7. [Medline].

  34. Gicquel C, Bertagna X, Le Bouc Y. Recent advances in the pathogenesis of adrenocortical tumours. Eur J Endocrinol. Aug 1995;133(2):133-44. [Medline].

  35. Gicquel C, Bertherat J, Le Bouc Y, Bertagna X. Pathogenesis of adrenocortical incidentalomas and genetic syndromes associated with adrenocortical neoplasms. Endocrinol Metab Clin North Am. Mar 2000;29(1):1-13, vii. [Medline].

  36. Golden SH, Robinson KA, Saldanha I, Anton B, Ladenson PW. Clinical review: Prevalence and incidence of endocrine and metabolic disorders in the United States: a comprehensive review. J Clin Endocrinol Metab. Jun 2009;94(6):1853-78. [Medline].

  37. Hamrahian AH, Ioachimescu AG, Remer EM, et al. Clinical utility of noncontrast computed tomography attenuation value (hounsfield units) to differentiate adrenal adenomas/hyperplasias from nonadenomas: Cleveland Clinic experience. J Clin Endocrinol Metab. Feb 2005;90(2):871-7.

  38. Juarez D, Brown RW, Ostrowski M, et al. Pheochromocytoma associated with neuroendocrine carcinoma. A new type of composite pheochromocytoma. Arch Pathol Lab Med. Dec 1999;123(12):1274-9. [Medline].

  39. Kasperlik-Zaluska AA. Clinical results of the use of mitotane for adrenocortical carcinoma. Braz J Med Biol Res. Oct 2000;33(10):1191-6. [Medline].

  40. 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. Oct 2000;11(10):1281-7. [Medline].

  41. Kloos RT, Gross MD, Francis IR, et al. Incidentally discovered adrenal masses. Endocr Rev. Aug 1995;16(4):460-84. [Medline].

  42. Kloos RT, Korobkin M, Thompson NW, et al. Incidentally discovered adrenal masses. Cancer Treat Res. 1997;89:263-92. [Medline].

  43. Koch CA, Pacak K, Chrousos GP. The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab. Dec 2002;87(12):5367-84. [Medline].

  44. Korobkin M, Brodeur FJ, Francis IR, et al. CT time-attenuation washout curves of adrenal adenomas and nonadenomas. AJR Am J Roentgenol. Mar 1998;170(3):747-52. [Medline].

  45. Latronico AC, Chrousos GP. Extensive personal experience: adrenocortical tumors. J Clin Endocrinol Metab. May 1997;82(5):1317-24. [Medline].

  46. Latronico AC, Chrousos GP. Neoplasms of the adrenal cortex. Clinical and basic aspects. Cancer Treat Res. 1997;89:217-37. [Medline].

  47. Latronico AC, Pinto EM, Domenice S, et al. An inherited mutation outside the highly conserved DNA-binding domain of the p53 tumor suppressor protein in children and adults with sporadic adrenocortical tumors. J Clin Endocrinol Metab. Oct 2001;86(10):4970-3. [Medline].

  48. Li BD, Douglass HO. Management of the incidentally discovered adrenal mass or "incidentaloma". J La State Med Soc. Aug 1997;149(8):291-9. [Medline].

  49. Linos DA. Management approaches to adrenal incidentalomas (adrenalomas). A view from Athens, Greece. Endocrinol Metab Clin North Am. Mar 2000;29(1):141-57. [Medline].

  50. Lubitz JA, Freeman L, Okun R. Mitotane use in inoperable adrenal cortical carcinoma. JAMA. Mar 5 1973;223(10):1109-12. [Medline].

  51. Lynch HT, Mulcahy GM, Harris RE, et al. Genetic and pathologic findings in a kindred with hereditary sarcoma, breast cancer, brain tumors, leukemia, lung, laryngeal, and adrenal cortical carcinoma. Cancer. May 1978;41(5):2055-64. [Medline].

  52. Lynch HT, Radford B, Lynch JF. SBLA syndrome revisited. Oncology. 1990;47(1):75-9. [Medline].

  53. Mantero F, Arnaldi G. Investigation protocol: adrenal enlargement. Clin Endocrinol (Oxf). Feb 1999;50(2):141-6. [Medline].

  54. Mantero F, Arnaldi G. Management approaches to adrenal incidentalomas. A view from Ancona, Italy. Endocrinol Metab Clin North Am. Mar 2000;29(1):107-25, ix. [Medline].

  55. Nader S, Hickey RC, Sellin RV, Samaan NA. Adrenal cortical carcinoma. A study of 77 cases. Cancer. Aug 15 1983;52(4):707-11. [Medline].

  56. Pimentel M, Johnston JB, Allan DR, et al. Primary adrenal lymphoma associated with adrenal insufficiency: a distinct clinical entity. Leuk Lymphoma. Jan 1997;24(3-4):363-7. [Medline].

  57. Reincke M. Mutations in adrenocortical tumors. Horm Metab Res. Jun-Jul 1998;30(6-7):447-55. [Medline].

  58. Rossi R, Tauchmanova L, Luciano A, et al. Subclinical Cushing's syndrome in patients with adrenal incidentaloma: clinical and biochemical features. J Clin Endocrinol Metab. Apr 2000;85(4):1440-8. [Medline].

  59. Saeger W. Pathology of adrenal neoplasms. Minerva Endocrinol. Mar 1995;20(1):1-8. [Medline].

  60. Salvatore JR, Ross RS. Primary bilateral adrenal lymphoma. Leuk Lymphoma. Jun 1999;34(1-2):111-7. [Medline].

  61. Sawin RS. Functioning adrenal neoplasms. Semin Pediatr Surg. Aug 1997;6(3):156-63. [Medline].

  62. Schteingart DE. Management approaches to adrenal incidentalomas. A view from Ann Arbor, Michigan. Endocrinol Metab Clin North Am. Mar 2000;29(1):127-39, ix-x. [Medline].

  63. Schulick RD, Brennan MF. Adrenocortical carcinoma. World J Urol. Feb 1999;17(1):26-34. [Medline].

  64. Schwartz RW, Sloan DA, Kenady DE. Diagnosis and treatment of primary adrenal tumors. Curr Opin Oncol. Feb 1991;3(1):121-7. [Medline].

  65. Shimshi M, Ross F, Goodman A, Gabrilove JL. Virilizing adrenocortical tumor superimposed on congenital adrenocortical hyperplasia. Am J Med. Sep 1992;93(3):338-42. [Medline].

  66. Soffen EM, Solin LJ, Rubenstein JH, Hanks GE. Palliative radiotherapy for symptomatic adrenal metastases. Cancer. Mar 15 1990;65(6):1318-20. [Medline].

  67. Soon PS, Sidhu SB. Molecular basis of adrenocortical carcinomas. Minerva Endocrinol. Jun 2009;34(2):137-47. [Medline].

  68. Soussi T, Leblanc T, Baruchel A, Schaison G. Germline mutations of the p53 tumor-suppressor gene in cancer-prone families: a review. Nouv Rev Fr Hematol. Feb 1993;35(1):33-6. [Medline].

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

  70. Strosberg JR, Hammer GD, Doherty GM. Management of adrenocortical carcinoma. J Natl Compr Canc Netw. Jul 2009;7(7):752-8; quiz 759. [Medline].

  71. Sullivan M, Boileau M, Hodges CV. Adrenal cortical carcinoma. J Urol. Dec 1978;120(6):660-5. [Medline].

  72. Tauchmanova L, Rossi R, Biondi B, et al. Patients with subclinical Cushing's syndrome due to adrenal adenoma have increased cardiovascular risk. J Clin Endocrinol Metab. Nov 2002;87(11):4872-8. [Medline].

  73. Traill Z, Tuson J, Woodham C. Adrenal carcinoma in a patient with Gardner's syndrome: imaging findings. AJR Am J Roentgenol. Dec 1995;165(6):1460-1. [Medline].

  74. Udelsman R, Fishman EK. Radiology of the adrenal. Endocrinol Metab Clin North Am. Mar 2000;29(1):27-42, viii. [Medline].

  75. Wakatsuki S, Sasano H, Matsui T, et al. Adrenocortical tumor in a patient with familial adenomatous polyposis: a case associated with a complete inactivating mutation of the APC gene and unusual histological features. Hum Pathol. Mar 1998;29(3):302-6. [Medline].

  76. Wei CY, Chen KK, Chen MT, et al. Adrenal cortical carcinoma with tumor thrombus invasion of inferior vena cava. Urology. Jun 1995;45(6):1052-4. [Medline].

  77. Weingartner K, Gerharz EW, Bittinger A, et al. Isolated clinical syndrome of primary aldosteronism in a patient with adrenocortical carcinoma. Case report and review of the literature. Urol Int. 1995;55(4):232-5. [Medline].

 
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