Complete Radiological Response of Recurrent Metastatic Adrenocortical Carcinoma to Pembrolizumab and Mitotane

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SAGE Publications Ltd

Abstract

Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Treatment options for ACC are limited, with resection the main intervention. Most cases present in late metastatic cases, and data regarding effective therapies is limited. We report a case of ACC in a 40-year-old woman with history of ACC postadrenalectomy, who presented with recurrent metastatic ACC in the left perinephric space. She was started on pembrolizumab which was added to her mitotane maintenance therapy. Complete radiological response was achieved after 4 cycles of pembrolizumab. As far as we know, this is the first case to achieve complete radiological response with mitotane and pembrolizumab in recurrent metastatic ACC, with negative prognostic markers and no prior radiotherapy. As our findings are in the setting of one clinical case, we suggest the need to perform a trial to assess the benefit of combining mitotane and pembrolizumab in treating metastatic ACC. © The Author(s) 2021.

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Acc, Adrenocortical carcinoma, Complete radiological response, Immunoscore, Mitotane, Pembrolizumab, Regulatory t-cells, Tumor-associated macrophages, Fluorodeoxyglucose, Adrenal cortex carcinoma, Adult, Article, Cancer localization, Cancer radiotherapy, Cancer surgery, Case report, Cd3+ t lymphocyte, Cd8+ t lymphocyte, Clinical article, Clinical feature, Disease severity, Drug efficacy, Drug safety, Female, Gene amplification, High throughput sequencing, Human, Human tissue, Maintenance therapy, Multiple cycle treatment, Nuclear magnetic resonance imaging, Patient history of adrenalectomy, Positron emission tomography-computed tomography, Recurrent metastatic adrenal cortex carcinoma, Regulatory t lymphocyte, Treatment response, Tumor growth, Tumor volume, Tumor-associated macrophage

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