EDP-M plus sintilimab in the treatment of adrenocortical carcinoma: a case report

Background: Adrenocortical carcinoma (ACC) is a rare malignancy occurring in the adrenal cortex characterized by its low incidence rate, aggressive tumor behavior with high propensity of invasion and distant metastasis, and poor prognosis. When detected, it usually appears as advanced tumor with limited treatment options. Systemic chemotherapy is the only recommended treatment option and EDP-M plus sintilimab has recently emerged as a new treatment option. In the present study, we report a case of ACC treated with EDP-M plus sintilimab. Case Description: We present a 30-year-old female patient with ACC and bilateral lung metastases. PET-CT scan showed multiple metastatic sites in both lungs, among which the largest one was located in the lower lobe of the right lung, with a size of 42 mm × 22 mm. A space-occupying lesion was also found in the right adrenal gland with a size of 92 mm × 51 mm. The patient was treated with EDP plus sintilimab for four cycles in our hospital and mitotane was introduced after the first cycle. Follow-ups after the second and fourth cycles found significantly reduced lung metastases with all imaging examinations indicating PR status. The patient received maintenance therapy thereafter with sintilimab plus mitotane. Until recently, the patient's lung metastases have basically disappeared and no disease progression has been observed. The progression free survival (PFS) of this patient has been extended to about 7 months. Conclusions: Although deemed as the standard chemotherapeutic regimen for advanced ACC, the efficacy of EDP-M is not satisfactory. This case study demonstrates the clinical effectiveness of EDP-M plus sintilimab in the treatment of advanced ACC with good patient tolerance, suggesting that this regimen combination can be a promising treatment option for refractory ACCs.