ACC patients with progressive disease that cannot be addressed with radiation, surgery or interventional radiology often turn to systemic therapy (targeted drugs and chemotherapy). Given the lack of any approved drugs for ACC, nor a consensus on the most effective drugs, clinical trials of systemic therapy may be appropriate in consultation with a patient's physician. Fortunately, there has been substantial activity in exploring new drugs in ACC in clinical trials.

Report on the Recent Axitinib Study - At the recent Annual Meeting of the American Society of Clinical Oncology (ASCO), a poster presented the preliminary findings of the phase II study of Axitinib in ACC. Axitinib is a drug that targets VEGFR, PDGFR and CKIT. The abstract may be viewed at http://abstracts.asco.org/144/AbstView_144_134887.html. Of 33 ACC patients with progressive disease, 1 patient had a partial response (substantial tumor shrinkage) and 10 patients had disease stabilization for 6 months or longer. These results are modestly encouraging in the context of past ACC clinical trials:

Closing of the Recent Dovitinib Study - The Canadian phase II study of Dovitinib recently completed enrollment of its 20 ACC patients. Dovitinib is a drug that targets FGFR, VEGFR and PDGFR. Prior phase II studies at the University of Virginia and Seoul National University have been accrued fully, so there are no phase II Dovitinib studies enrolling ACC patients at the moment. Preliminary results from the University of Virginia were reported at last year's ASCO Annual Meeting: http://meetinglibrary.asco.org/content/114871-132. The preliminary results were quite encouraging, although further analysis of the data from all three studies will be needed to determine whether additional studies of Dovitinib in ACC will be initiated. 

Opening of the New Regorafenib Study - Memorial Sloan-Kettering recently opened a phase II study of Regorafenib, a drug that targets FGFR, VEGFR and PDGFR (similar to Dovitinib). The estimated enrollment at the New York, NY location will be 32 ACC patients.  

Opening of the New Pembrolizumab (MK-3475) Study - Merck Pharmaceuticals recently opened an arm of a phase IB study of Pembrolizumab (MK-3475) specifically for salivary gland cancer patients (including ACC patients). The drug targets PD-1, an immune checkpoint that can restrain the immune system's attacks on tumors. The study has 18 sites open across the USA, Europe, Canada, Korea and Japan. 

Updated Phase I Studies - Clinical trials that recruit ACC patients in particular may not be convenient or appropriate for all ACC patients. Therefore, ACCRF also lists selected phase I studies on its Current Studies webpage that enroll patients with multiple tumor types. The studies involve drugs that inhibit targets suspected of contributing to the progression of ACC, such as FGFR, IGF-1R and TRK. Two of the studies combine an approved FGFR inhibitor (Pazopanib) with HDAC inhibitors (Vorinostat and Abexinostat), two classes of drugs that have shown activity in significant subsets of ACC patients.   

Options for Patients with NOTCH-Mutated Tumors - Most ACC tumors have similar biology in that the MYB gene is overly active. However, each ACC tumor has its own molecular profile with its own alterations. A small but significant subset of ACC tumors have been found to harbor mutations in NOTCH pathway genes. For patients who have had tumor profiling that identified a NOTCH alteration, ACCRF has listed at the bottom of its Current Studies webpage some phase I studies of NOTCH inhibitors that may be appropriate.  

In all cases, ACC patients who are considering clinical trials should discuss their situations and options with their physicians.