Systemic Therapy and Clinical Trials for ACC patients

Systemic Therapy refers to drugs that travel through the bloodstream to reach cancer cells throughout the body, and includes chemotherapy, targeted drugs and immunotherapy. No systemic therapy has been approved by the U.S. Food and Drug Administration (FDA) for ACC because non has been effective across 'large groups' of ACC patients.  However, ACC patients with advanced disease may resort to systemic therapy when dealing with growing tumors that are numerous or located near vital organs that are no longer treatable with conventional treatments such as surgery, radiation, ablation therapy, etc. 

Patients may seek systemic therapies by means of a clinical trial.  Clinical trials are research studies that test how well new medical approaches work in people with diseases or cancers such as ACC. Fortunately, research has led the way in which more clinical trials are available for ACC patients.  Many of these clinical trials can be found here

There are a number of drugs that may comprise a clinical trial for ACC patients.  
Targeted agents are drugs whose activity is intended to interfere with specific molecules involved in cancer progression.  They may block cell growth signals, boost the immune system, stop the growth of blood vessels that support tumors, deliver toxic drugs to cancers or fix cell processes that would normally suppress tumors.  Targeted agents can be less toxic than chemotherapy which is another from of systemic therapy. 

Chemotherapy kills all rapidly dividing cells, including tumors and normal healthy cells in the blood, bone marrow, hair follicles and more.  For this reason, chemotherapy may cause more severe side-effects with a combination of fatigue, hair loss, nausea, damage to the heart, liver and kidneys.  Chemotherapy may be an option for more aggressive forms of ACC cells in which the cells divide quickly. 

Immunotherapy encompasses various approaches that harnesses a patient's immune system to attack tumor cells.  Checkpoint inhibitors that target immune system molecules (PD-1, PD-L1 and CTLA) have not done well in ACC clinical trials although a small portion of patients have done well.  Other types of immunotherapies include vaccines and engineered cell therapies. 

Today, we may see clinical trials combining drugs such as those explained above. 

Tumor Profiling is becoming increasingly important in helping physicians to select the best from of systemic therapy for their patients.  Tumor profiling is the process of scanning the DNA, RNA or protein from a patient's tumor to understand what molecules are driving the growth of their tumor.  Tumor profiling reports may be ordered by a medical oncologist and may help in identifying specific drugs that target these specific molecules.  Patients can learn more about tumor profiling here   

Patients should seek the advice of a multidisciplinary team of surgeons, radiation oncologists and medical oncologist throughout each stage of their cancer journey.