Oligometastatic adrenocortical carcinoma: definition and treatment

Purpose of review: Oligometastatic adrenocortical carcinoma (ACC) represents a distinct clinical subset of metastatic disease characterized by a limited tumor burden and potentially indolent biology. This review summarizes current evidence on its definition and management strategies. Recent findings: Although mitotane and EDP-M chemotherapy remain the backbone of systemic therapy for advanced ACC, increasing evidence supports integrating local treatments - such as surgery, stereotactic body radiotherapy (SBRT), image-guided thermal ablation (IGT), and transarterial embolization (TACE/TARE) - in selected patients. Retrospective studies suggest that individuals with ≤5 metastases or lesions <3 cm, often classified as stage IVa, achieve higher disease control rates and prolonged survival when local and systemic therapies are combined. Decision-making should consider patient fitness, tumor biology (Ki-67 index, time to recurrence), and prior treatments within a multidisciplinary framework. Summary: If a definition of oligometastatic ACC is required, a reasonable one would include stage IVa disease or up to five metastases <3 cm. Management should rely on a multidisciplinary approach in referral centers, integrating systemic and local therapies to optimize survival and quality of life.