eMedicine Specialties > Oncology > Carcinomas of Endocrine Organs

Adrenal Carcinoma: Follow-up

Author: Gabriel I Uwaifo, MBBS, Clinical and Research Attending, Assistant Professor of Medicine and Endocrinology, MedStar Clinical Research Center, The MedStar Research Institute and the Washington Hospital Center
Coauthor(s): Antonio Tito Fojo, MD, Senior Clinical Investigator, Medicine Branch, Division of Cancer Treatment, National Cancer Institute, National Institutes of Health
Contributor Information and Disclosures

Updated: May 22, 2006

Follow-up

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

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 nonfunctional variants that invariably present when they are very large or when they 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.
  • 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. A report from Icard and associates suggests that tumor prognosis also may be influenced by the hormonal product of the tumor, but this has not been replicated in other series. The mitotic index, the presence of atypical mitoses on histologic analysis, a tumor mass larger than 250 g, and tumor diameter larger than 10 cm are all indices of a poor prognosis.
  • 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.
  • 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.

Miscellaneous

Medicolegal Pitfalls

  • Because the management of AC is still undergoing considerable flux, significant controversy exists and very few, if any, universally accepted standards have been determined. Until a consensus conference on AC is convened where the recent developments in the field and accumulated clinical experience of the last few decades are integrated, only broad guidelines can be suggested.
  • A full evaluation is advised in all patients with a distinct adrenal nodule or tumor larger than 1 cm in order to determine whether the tumor is functional. The general agreement is that all functional masses should be removed.
  • No definitive guidelines exist for all nonfunctional adrenal masses being followed serially. A suggested follow-up regimen is to perform repeat adrenal CT scans or MRI scans 3-6 months after the initial evaluation, then yearly (some suggest every 6 mo for the first few years) in order to detect any change in tumor size. Accompany these with periodic checks of hormonal profiles (after 1 y, then every 1-2 y thereafter).
  • Removal of all nonmetastatic adrenal masses larger than 6 cm is advisable (several authorities say 5 cm, while others say 4 cm), regardless of the patient's hormonal profile.
  • Use a higher index of suspicion for children with adrenal masses; these may be malignant even when smaller than 4-5 cm in diameter.
 


More on Adrenal Carcinoma

Overview: Adrenal Carcinoma
Differential Diagnoses & Workup: Adrenal Carcinoma
Treatment & Medication: Adrenal Carcinoma
Follow-up: Adrenal Carcinoma
References

References

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

  2. 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].

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

  4. 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].

  5. 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].

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

  7. 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].

  8. 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].

  9. 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.

  10. 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].

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

  12. 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].

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

  14. 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].

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

  16. 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].

  17. 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].

  18. 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.

  19. 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].

  20. 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].

  21. 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].

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

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

  24. 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].

  25. 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].

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

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

  28. 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].

  29. 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].

  30. 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].

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

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

  33. 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].

  34. 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].

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

  36. McNicol AM. Molecular aspects of adrenal tumours. Minerva Endocrinol. Mar 1995;20(1):9-13. [Medline].

  37. 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].

  38. 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].

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

  40. 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].

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

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

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

  44. 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].

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

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

  47. 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].

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

  49. 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].

  50. 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].

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

  52. 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].

  53. 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].

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

  55. 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].

  56. 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].

  57. 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].

Further Reading

Keywords

adrenal carcinoma, AC, adrenocortical carcinoma, adrenal cancer, adrenocortical cancer, primary adrenocortical malignancies, malignant adrenocortical neoplasms, malignant adrenal tumors, malignant adrenocortical tumors, adrenocortical masses, adrenal incidentalomas

Contributor Information and Disclosures

Author

Gabriel I Uwaifo, MBBS, Clinical and Research Attending, Assistant Professor of Medicine and Endocrinology, MedStar Clinical Research Center, The MedStar Research Institute and the Washington Hospital Center
Gabriel I Uwaifo, MBBS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, American Society of Hypertension, and Endocrine Society
Disclosure: Nothing to disclose.

Coauthor(s)

Antonio Tito Fojo, MD, Senior Clinical Investigator, Medicine Branch, Division of Cancer Treatment, National Cancer Institute, National Institutes of Health
Disclosure: Nothing to disclose.

Medical Editor

Michael C Perry, MD, Professor, Department of Internal Medicine, Nellie B Smith Chair of Oncology, Director, Division of Hematology and Oncology, University of Missouri at Columbia/Ellis Fischel Cancer Center
Michael C Perry, MD 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, Missouri State Medical Association, Southern Association for Oncology, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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, American Society for Therapeutic Radiology and Oncology, and American Society of Clinical Oncology
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, 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

John S Macdonald, MD, Professor of Medicine, New York Medical College; Chief, Division of Medical Oncology, St Vincent's Hospital and Medical Center; Medical Director, Saint Vincent's Comprehensive Cancer Center
Disclosure: Nothing to disclose.

 
 
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