Primary tumour resection in metastasised adrenocortical carcinoma

Up to 30% of adrenocortical carcinoma (ACC) patients have metastasised disease upon initial presentation, and systemic treatments currently fail to sufficiently improve survival. Palliative primary tumour resection can be considered for symptomatic relief, but its potential survival benefit remains a topic of debate. This systematic review therefore aims to assess the effect of primary tumour resection on overall survival in patients with metastatic ACC. A systematic review was performed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Relevant databases were searched from 2000 to 2024 for studies on primary tumour resection in metastatic ACC. Overall survival data were analysed. A total of 13 studies on primary tumour resection for metastatic ACC were included. All studies were retrospective and assessed as having a high risk of bias. Data regarding adequate patient characteristics and indications for surgery were missing in all studies. Hence, the current literature is hampered by both indication and selection biases to draw any conclusions on the survival benefit of primary tumour resection in patients with metastasised ACC. However, 12 out of 13 studies (92%) demonstrated longer overall survival after primary tumour resection compared to no surgery. Whereas this is in line with retrospective data on other cancers, randomised controlled trials in other tumours, such as breast and colorectal cancers, have failed to display survival benefits of primary tumour resection. These cancers are, however, relatively chemo-sensitive, unlike ACC. Primary tumour resection could therefore only be considered on an individual patient basis.