AUTHOR=Weng Yan , Wang Lin , Wang Xiao-Yi , Fan Xin-Xiang , Yan Li , Li Zhi-Hua , Zhang Shao-Ling 

TITLE=Case report: Remarkable response to a novel combination of mitotane, etoposide, paraplatin, and sintilimab in a patient with metastatic adrenocortical carcinoma

JOURNAL=Frontiers in Endocrinology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2023.1115893

DOI=10.3389/fendo.2023.1115893

ISSN=1664-2392

ABSTRACT=Background: Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis and limited treatment options for metasta ses. However, new effective regimens are emerging for specific conditions in metastatic ACC.We report a case of a 36-year-old male diagnosed with metastatic ACC, who had a large left adrenal mass (158 mm×112 mm), and multiple metastases in the liver and lungs. Genetic testing revealed a microsatellite instability-high (MSI-H) tumor, a splice mutation in MLH1, and a high tumor mutational burden (TMB). After the left adrenalectomy, he received sequential treatment with a combination of mitotane, etoposide, paraplatin (EP-M), and sintilimab. His condition has been assessed as stable disease since the sixth cycles of the combined regimen.This case highlights the remarkable response of our patient's ACC with MSI-H tumor, MLHI spice mutation, and high TMB to treatment with a novel combination of EP-M and sintilimab. Our findings suggest a promising therapeutic option for patients with similar molecular profiles .