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Adrenocortical carcinoma is a rare tumor that affects only 1 to 2 persons per one million population. It usually occurs in adults, and the median age at diagnosis is 44 years. Although adrenal carcinoma is potentially curable at early stages, only 30% of these malignancies are confined to the adrenal gland at the time of diagnosis. Radical surgical excision is the treatment of choice for patients with localized malignancies and remains the only method by which long-term disease-free survival may be achieved. Overall 5-year survival for tumors resected for cure is approximately 40%.
Retrospective studies have identified two important prognostic factors: completeness of resection and stage of disease. Patients without evidence of invasion into local tissues or spread to lymph nodes have an improved prognosis. The role of DNA ploidy as a prognostic indicator is controversial, with some  studies, such as ECOG-1879 for example, showing correlation between aneuploidy and prognosis, and other studies [2,4] showing no correlation.
Approximately 60% of patients present with symptoms related to excessive hormone secretion, but hormone testing reveals that 60% to 80% of tumors are functioning.[5,6] Nonfunctioning carcinomas may be heralded by symptoms of local invasion by tumor or by metastases. Initial evaluation should include, in addition to appropriate endocrine studies, computed tomography and/or magnetic resonance imaging of the abdomen. Selective angiography and adrenal venography may be helpful in identifying smaller lesions and for distinguishing tumors of the adrenal gland from tumors of the upper pole of the kidney. Although the use of positron emission tomography may be effective in identifying unsuspected sites of metastases, its role as a staging tool is unclear. The detection of metastatic lesions may allow effective palliation of both functioning and nonfunctioning lesions.
The most common sites of metastases are the peritoneum, lung, liver, and bone. Palliation of metastatic functioning tumors may be achieved by resection of both the primary tumor and metastatic lesions. Unresectable or widely disseminated tumors may be palliated by antihormonal therapy with mitotane, systemic chemotherapy, or (for localized lesions) radiation therapy. However, survival for patients with stage IV tumors is usually less than 9 months unless a complete remission is achieved.[6,8,9,10] There is no convincing evidence to date that systemic therapy will improve the survival duration of patients with adrenal cancer.
Adrenocortical carcinoma can be classified as follows:
Note: The American Joint Committee on Cancer has recently published a new edition of the AJCC Cancer Staging Manual, which includes revisions to the staging for this disease. The PDQ Adult Treatment Editorial Board, which is responsible for maintaining this summary, is currently reviewing the new staging to determine the changes that need to be made in the summary. In addition to updating this Stage Information section, additional changes may need to be made to other parts of this summary to ensure that it is up-to-date. The changes will be made as soon as possible.
The stage of adrenocortical carcinoma is determined by the size of the primary tumor, the degree of local invasion, and whether it has spread to regional lymph nodes or distant sites.[1,2,3,4] Proper staging should include computed tomography (CT) of the abdomen. Magnetic resonance imaging (MRI) may add specificity to CT evaluation of an adrenal mass. In-phase and out-of-phase T1-weighted imaging may be the most effective noninvasive method to differentiate benign from malignant adrenal masses. MRI can also often clearly demonstrate any evidence of extracapsular tumor invasion, extension into the vena cava, or metastases. Patency of surrounding vessels can often be demonstrated with gadolinium-enhanced sequences or flip-angle techniques. Vena caval contrast studies and angiography may provide additional staging information and allow for a more complete preoperative assessment. A review of published data from 608 patients revealed the following stage distribution at diagnosis: 3% stage I, 29% stage II, 20% stage III, and 49% stage IV.
Stages are defined by TNM classification.
Lymph nodes (N)
Standard treatment options:
Treatment options under clinical evaluation:
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I adrenocortical carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage II adrenocortical carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III adrenocortical carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Temporary palliation of disseminated adrenocortical carcinomas can sometimes be achieved with the chemotherapeutic agent mitotane. Although measurable partial remissions are unusual and are reported in only 19% to 34% of cases, excellent palliation of hormone symptoms is commonly observed. Prolonged treatment with mitotane, however, is often limited by gastrointestinal and neurologic toxicity. Local recurrences and selected sites of metastatic disease can sometimes be palliated surgically.
Clinical trials such as ECOG-1879 and SWOG-8325, especially phase I and II trials that evaluate newer chemotherapeutic and biological agentsare appropriate and should be considered whenever possible.[3,4,5,6] Palliative chemotherapy with cisplatin-based regimens has produced objective responses in approximately 30% of patients treated.[4,5,7,8] One study reported that doxorubicin produced objective responses in 3 of 16 patients with poorly differentiated, nonhormone-producing tumors but no responses in 15 patients whose disease did not respond to mitotane. Use of both platinum-based chemotherapy and mitotane achieved a 48.6% objective response and median time-to-progression of 18 months in responders.[Level of evidence: 3iiDiv] In 10 of 72 patients, subsequent surgical resection was possible.
Treatment options under clinical evaluation:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage IV adrenocortical carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
The question and selection of further treatment for patients with adrenocortical carcinoma depends on many factors, including previous treatment and site of recurrence as well as individual patient considerations. Local recurrence and selected sites of metastatic disease can sometimes be palliated by surgery. Although none of these patients can be considered curable, palliation of hormonal symptoms and occasional 5-year survivals can be achieved.[1,2] Substantial morbidity, however, is associated with resection of these recurrent tumors. Clinical trials, such as ECOG-1879 and SWOG-8325,are appropriate and should be considered whenever possible, especially phase I and II trials that evaluate newer chemotherapeutic and biological agents.[3,4,5,6,7]
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent adrenocortical carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adrenocortical carcinoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewer for Adrenocortical Carcinoma Treatment is:
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Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
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National Cancer Institute: PDQ® Adrenocortical Carcinoma Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://cancer.gov/cancertopics/pdq/treatment/adrenocortical/HealthProfessional. Accessed <MM/DD/YYYY>.
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Last Revised: 2008-05-16
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