Context: The natural history and malignant potential of cases classified as atypical adrenal masses (AAMs) are unknown.
Objective: To describe the radiological characteristics and clinical outcomes of AAMs.
Design And Participants: A multicenter retrospective study.
Patients And Methods: The primary endpoints were objective response rate (ORR) and disease control rate (DCR). Secondary endpoints were duration of response, blood pressure control, safety, overall and progression-free survival rates, MIBG uptake, and correlations with genetic background.
Results: The study included 25 patients.
Adrenocortical carcinoma (ACC) is a rare cancer with high recurrence rates and heterogeneous clinical behavior. The role of adjuvant therapy remains unclear because of the challenges in collecting high-quality data for a rare cancer. The current treatment recommendations and guidelines for adjuvant therapy are mostly derived retrospectively from national databases and the treatment outcomes of patients seen in referral centers.
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