Recurrence or Metastasis

ACC patients with recurrent disease or those who have metastases at their initial diagnosis face an uncertain prognosis and many potential treatment paths. Physicians do not have a clear standard of care to recommend, so treatment plans are highly individualized and patient preferences often drive the final decision.

Two key factors tend to frame the decision for most patients:

1. Tumor growth rate – Is the disease stable or do imaging studies show clear growth over the past 3-6 months?
2. Tumor extent and location – How numerous and large are the tumors? Are they near vital structures such as the spine, bronchus, brain or heart?

Patients with stable disease

ACC growth rates can be highly variable. Patients with lung metastases may go years or even decades without experiencing any recognizable growth. There are even documented cases of spontaneous regression where metastases have disappeared without treatment.

Given the limited effectiveness and substantial side effects of surgery, radiation and systemic therapies, many doctors will recommend that ACC patients with stable disease follow a strategy of “watchful waiting”. Such patients are closely monitored until such time as there is progressive disease (clear tumor growth or new metastases) at which point new treatments will be considered.

A patient’s personal preferences may lead to a more aggressive strategy that involves stereotactic body radiation therapy (SBRT) or interventional radiology (such as radiofrequency ablation or brachytherapy).

Usually, systemic therapy (chemotherapy and targeted drugs) is not chosen because no drugs have been found yet to be effective in a significant subset of ACC patients. Extensive research is underway that may provide attractive options in the future for patients with stable disease.

Patients with progressive disease

If you have tumors that are clearly growing, you face a journey with few signposts and even more potential paths to follow. The extent and location of the ACC tumors will help guide the decisions that you and your doctor come to.

If the tumors are few, well-defined and don’t encroach on vital organs, then the options of stereotactic body radiation therapy (SBRT), interventional radiology or, in rare cases, surgery may be appropriate. The treatments may not be curative, but they may lead to disease stabilization.

In many situations, physicians recommend to patients with multiple growing recurrences that systemic therapy is necessary to address the evident and as-yet-unidentified tumors. This is particularly the case if the tumors are near vital structures that limit the scope of radiation, interventional radiology or surgery to address the problem.

Given the limited effectiveness of chemotherapy in most ACC patients, doctors often point to the option of clinical trials of targeted drugs. Of course, the attractiveness of any clinical trial will depend upon the scientific promise of the drug, the patient’s ability to travel, insurance coverage, prior treatments and personal preferences.

The following table provides a framework for thinking about the options that many patients choose depending upon their situation.

Patients may want to read and share with their doctors the Guide to Systemic Therapy for ACC Patients that reviews the history of ACC clinical trials and the trend towards personalized medicine. More information on current ACC clinical trials as well as new research is available on this site.

In all cases, it is prudent for patients to consult with a multi-disciplinary team of ACC specialists (surgeons, radiation oncologists and medical oncologists) before selecting the best course of action for their particular situation.