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Would you offer immunotherapy first line for patients with adrenocortical carcinoma outside of a clinical trial?

The phase 2 trial of pembrolizumab in JCO 2019 showed efficacy, and I have patients preferring to avoid chemotherapy.
1 Answer
Mednet MemberInvited Expert
Medical Oncology · Mayo Clinic
Answered on · Updated on

I have not been using ICI as first-line therapy for ACC unless there are concerns for tolerability to EDP. We also do usually not include mitotane in EDP in patients with nonfunctional ACCs. Given the somewhat promising activity of pembrolizumab in ACC, it is a very reasonable second-line choice as well as an option when EDP is not feasible; but all who have given EDP know how difficult that regimen is, both in terms of AEs and logistically. I would love to see more data on ICI as first-line therapy.

A recently reported phase II trial (28% without prior systemic therapy) of pembrolizumab reported ORR of 23% and although the median PFS was short for the group as a whole, 2.1 months, the responses were durable in some patients. 

As stated, mitotane is a very challenging drug to use and although I may prescribe it, involvement by endocrinology is essential given the adrenolytic properties. Co-management is key for all of my patients but some institutions (including ours) are fortunate to have dedicated endocrine oncologists trained in both endocrinology and oncology.