Abstract
Adrenocortical carcinoma (ACC) is a rare epithelial neoplasm, with a high tendency for local invasion and distant metastases, with limited treatment options. Surgical treatment is the method of choice. For decades, the mainstay of pharmacological treatment has been the adrenolytic drug mitotane, in combination with chemotherapy. Immunotherapy is the latest revolution in cancer therapy, however preliminary data with single immune checkpoint inhibitors showed a modest activity in ACC patients. The anti-neoplastic activity of immune checkpoint inhibitors such as anti-cytotoxic-T-lymphocyte-associated-antigen 4 (anti-CTLA-4), anti-programmed death-1 (anti-PD-1), and anti-PD-ligand-1 (PD-L1) antibodies in different solid tumors has aroused interest to explore the potential therapeutic effect in ACC as well. Multiple ongoing clinical trials are currently evaluating the role of immune checkpoint inhibitors in ACC (pembrolizumab, combination pembrolizumab and relacorilant, nivolumab, combination nivolumab and ipilimumab). The primary and acquired resistance to immunotherapy continue to counter treatment efficacy. Therefore, attempts are made to combine therapy: anti-PD-1 antibody and anti-CTLA-4 antibody, anti-PD-1 antibody and antagonist of the glucocorticoid receptor. The inhibitors of immune checkpoints would benefit patients with antitumor immunity activated by radiotherapy. Immunotherapy is well tolerated by patients; the most frequently observed side effects are mild. The most common adverse effects of immunotherapy are skin and gastrointestinal disorders. The most common endocrinopathy during anti-CTLA treatment is pituitary inflammation and thyroid disorders.
Highlights
Immunotherapy is a treatment strategy based on the activation of a dormant and malfunctioning immune system that does not ensure proper recognition of cancer cells, which leads to the development of neoplastic disease
The combination of anti-TIM-3 with anti-PD-1 or anti-CTLA-4 shows promise for the improvement of current immunotherapy [42]. It appears that inhibitors of immune checkpoints would benefit patients with antitumor immunity activated by radiotherapy, which can induce an immunogenic cell death and promote the activation of the T cell response [49]
There is no doubt that chemotherapy in the treatment of patients with the most advanced cancers has reached or is approaching its limit, especially in Adrenocortical carcinoma (ACC) patients; new treatment concepts are urgently needed
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. The discovery of immunocompetent molecules that can inhibit the interaction of a cancer cell with antigen presenting cells or effector cells has resulted in great advances in oncology and oncohematology Immunotherapeutic agents such as cytokines stimulating the immune system or monoclonal antibodies directed against tumor-cell-surface receptors have been approved. Due to its remarkable effectiveness in the case of some types of cancer, this strategy has revolutionized the existing methods of immunotherapy This revolution in cancer therapy took place in a very short time and currently, several years after the start of research, monoclonal antibodies such as anti-cytotoxic T cell antigen 4 (anti-CTLA-4), anti-programmed cell death protein 1 (anti-PD-1) and anti-programmed cell death protein ligand 1 (anti-PD-L1). Are used in routine treatment or are undergoing the last stages of clinical trials in patients with various types of cancer [2]
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