Endocrine and Neuroendocrine Tumors Special Issue—Checkpoint Inhibitors for Adrenocortical Carcinoma and Metastatic Pheochromocytoma and Paraganglioma: Do They Work?

Camilo Jimenez, Gustavo Armaiz-Pena, Patricia L.M. Dahia, Yang Lu, Rodrigo A. Toledo, Jeena Varghese, Mouhammed Amir Habra

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

Abstract

Adrenocortical cancers and metastatic pheochromocytomas are the most common malignancies originating in the adrenal glands. Metastatic paragangliomas are extra-adrenal tumors that share similar genetic and molecular profiles with metastatic pheochromocytomas and, subsequently, these tumors are studied together. Adrenocortical cancers and metastatic pheochromocytomas and paragangliomas are orphan diseases with limited therapeutic options worldwide. As in any other cancers, adrenocortical cancers and metastatic pheochromocytomas and paragangliomas avoid the immune system. Hypoxia-pseudohypoxia, activation of the PD-1/PD-L1 pathway, and/or microsatel-lite instability suggest that immunotherapy with checkpoint inhibitors could be a therapeutic option for patients with these tumors. The results of clinical trials with checkpoint inhibitors for adrenocor-tical carcinoma or metastatic pheochromocytoma or paraganglioma demonstrate limited benefits; nevertheless, these results also suggest interesting mechanisms that might enhance clinical responses to checkpoint inhibitors. These mechanisms include the normalization of tumor vasculature, modifi-cation of the hormonal environment, and vaccination with specific tumor antigens. Combinations of checkpoint inhibitors with classical therapies, such as chemotherapy, tyrosine kinase inhibitors, radiopharmaceuticals, and/or novel therapies, such as vaccines, should be evaluated in clinical trials.

Original languageEnglish (US)
Article number467
JournalCancers
Volume14
Issue number3
DOIs
StatePublished - Feb 2022

Keywords

  • Adrenocortical cancer
  • Avelumab
  • Checkpoint inhibitors
  • Ipilimumab
  • Metastatic paraganglioma
  • Metastatic pheochromocytoma
  • Nivolumab
  • Pembrolizumab

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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