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Review
. 2025 Aug 29:19:11795549251364042.
doi: 10.1177/11795549251364042. eCollection 2025.

Frontiers in Systemic Therapy for Unresectable or Metastatic Adrenocortical Carcinoma: Harnessing Novel Therapeutic Approaches

Adam E Singer  1 , Nikhita Kathuria-Prakash  1 , David Shabsovich  1 , Lizette Garcia  1 , Leland Damron  2 , Allan J Pantuck  3 , Alexandra Drakaki  1
Affiliations
Review

Frontiers in Systemic Therapy for Unresectable or Metastatic Adrenocortical Carcinoma: Harnessing Novel Therapeutic Approaches

Adam E Singer et al. Clin Med Insights Oncol. .

Abstract

Adrenocortical carcinoma is a rare, aggressive endocrine malignancy with limited effective systemic therapy options and an overall poor prognosis for unresectable or metastatic disease. Chemotherapy and mitotane are the traditional systemic therapies of choice. More recently, as drug development in oncology has shifted away from chemotherapy, a host of therapeutic approaches targeting novel mechanisms of action has been studied in adrenocortical carcinoma. This review summarizes all nonchemotherapeutic approaches that have been and/or are currently being tested in clinical trials for the treatment of unresectable or metastatic adrenocortical carcinoma.

Keywords: Adrenocortical carcinoma; immune checkpoint inhibitors; radiopharmaceuticals; systemic therapy; tyrosine kinase inhibitors.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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