Diseases of the Adrenal Cortex: Adrenal Cancer

Treatment for Adrenal Cortical Carcinoma

Adrenal carcinoma may be curable if treated at an early stage. Radical surgical excision is the treatment of choice for cancers which have not spread to other organs. This remains the only method by which long-term cure may be achieved. Approximately 40% of all patients who undergo a radical resection of this cancer will be alive 5 years later.

A number of scientific studies have identified two important prognostic factors: [1] how complete the surgeon was able to remove the tumor and its surrounding tissues, and [2] the stage of disease (explained below). As could be expected, patients without invasion of the tumor into nearby tissues or spread to lymph nodes have an improved prognosis.

The most common organ to which adrenal cortical cancer spreads (distant metastases) are the liver and lung. Occasionally, patients can have a relief of some of their symptoms (and possibly even a slight increase in length of survival) if metastatic lesions which are producing hormones are surgically resected along with the primary adrenal tumor. Tumors which are unresectable because they have grown into important or vital structures nearby, or those which are not curable because they have spread throughout the body may be palliated (made to be less obvious and cause less symptoms) by anti-hormonal therapy with mitotane, systemic chemotherapy, or (for localized lesions) radiation therapy. Despite these treatments, survival for patients with stage IV tumors is usually less than 9 months. Chemotherapy has very little to add in the treatment of this cancer, and as of 1998, there is no convincing evidence that it will improve the survival duration of patients with adrenal cancer.

Information on Staging

When physicians talk about staging a cancer, they are describing a means by which the tumor can be characterized and therefore allow predictions about the best treatment options. This also provides a means to give estimates about prognosis. Like all cancers, the stage of adrenocortical carcinoma is determined by several factors: [1] the size of the adrenal tumor itself, [2] the degree of invasion into surrounding structures, and [3] whether it has spread to lymph nodes or distant organs. Staging usually includes computed tomography (CT scans) of the abdomen. Magnetic resonance imaging (MRI) may add some information to that found on the CT, and it may also demonstrate evidence of tumor invasion into surrounding tissues or extension into the vena cava.


Like most other cancers, the stage of adrenal cortical cancers are defined by the "TNM" classification. TNM stands for "Tumor", "lymph Nodes", and "Metastasis".

• Stage I = cancers < 5 cm (2 inches) without spread to lymph nodes or distant organs.
• Stage II = cancers > 5cm without spread to lymph nodes or distant organs.
• Stage III = cancers of any size which have spread to lymph nodes, but not distant organs, or, those tumors which are invading nearby tissues without involving lymph nodes or distant organs.
• Stage IV = cancers which are invading local tissues and have spread to nearby lymph nodes, or, cancers which are growing into surrounding vital structures / organs, or, cancers of any size which have spread to any other distant organ (metastasized).
  

Stage Distribution of Patients with Adrenal Cortical Cancer

• Stage I ~ 5%
• Stage II ~ 30%
• Stage III ~ 25%
• Stage IV ~ 40%
  
  

Treatment Options by Stage

• Stage I. The standard approach would be for complete surgical removal of the tumor.
  
• Stage II. Same as for Stage I.
  
• Stage III. Complete surgical removal of the tumor with removal of lymph node tissues in the area. These patients are at high risk for disease recurrence and should be considered for enrollment in a clinical trial (see below). For patients unable to undergo complete resection, drug therapy with mitotane (see below) is often helpful.
  
• Stage IV. Usually not candidates for surgical resection because of the size of the tumor and the fact that it has already spread to other organs of the body. Usually candidates for mitotane treatments.
  
  

Anti-Hormonal / Chemotherapy

Temporary relief of symptoms due to disseminated adrenocortical carcinomas can sometimes be achieved with the chemotherapeutic agent mitotane. Although measurable decreases in tumor size are quite uncommon (20-30%), excellent relief of symptoms (due to the excess hormones produced) is commonly observed. This antitumor drug averages 10 months of symptom relief. Approximately 80% of patients with hormone producing tumors will show significant reductions in hormone production. The drug is not usually used unless metastases are present on x-rays studies (e.g. CT scans) or the remaining tumor is producing measurable levels of hormone. There does not appear to be a role for mitotane therapy if the patient has undergone complete resection of the tumor. Prolonged treatment with mitotane is often limited by gastrointestinal and neurologic toxicity.

Because these tumors are aggressive and many times unresectable for cure, clinical trials are appropriate and should be considered whenever possible, especially trials that evaluate newer chemotherapeutic and biologic agents. If you want more information on clinical trials for this cancer, call the Cancer Information Service at the National Cancer Institute 1-800-4-CANCER (1-800-422-6237).

Recurrent Adrenal Cortical Cancer

This aggressive tumor will often recur months or years after successful surgical removal of all apparent cancer. What to do for these patients depends on many factors, including the type of previous treatment and the location of the recurrence. Individual patient considerations will also need to be considered as well including their desire for further surgery, and other underlying health conditions. Local recurrence and a few selected sites of metastatic disease can sometimes be removed surgically, and this is the preferred mechanism to deal with such recurrences. Although very few of these patients can be considered curable, relief of hormonal symptoms and occasional 5-year survivals can be achieved. This chance at prolonged survival comes at some risk, however, since reoperating on these tumors is technically difficult and often associated with complications.