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.

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