This protocol addresses patients with advanced adrenocortical carcinoma who present with limited metastatic spread — confined to intra-abdominal sites, extra-abdominal sites, or both — where the extent of disease makes local therapeutic intervention feasible.
The defining characteristic is the contained distribution of metastases at the time of diagnosis. When the number and location of lesions are limited, local control becomes a meaningful clinical objective.
The protocol distinguishes between patients where complete resection of all lesions appears achievable and those with extra-abdominal involvement where a combined local approach may be warranted — both situations are covered.
Management integrates local disease control — through surgical and ablative means — with systemic medical therapy initiated as early as clinically feasible. The specific combination, the criteria for selecting each local modality, and the sequence are detailed in the full protocol.
Achievement and maintenance of a therapeutic mitotane blood level guides ongoing management; the target concentration range is specified in the full protocol.
DOI: 10.1530/EJE-18-0608
For patients presenting at time of initial diagnosis with limited intra-abdominal metastases we suggest surgical therapy if complete resection of all lesions seems feasible (+OOO).
In case of limited extra-abdominal lesions, we suggest adrenal tumor resection in conjunction with therapy aiming at long-term tumor control of the other lesions (+OOO).
The panel is convinced that in addition to surgery other local therapeutic measures (e.g. radiation therapy, radiofrequency ablation, cryoablation, microwave ablation, chemoembolization) are of value for therapy of advanced ACC.
In all patients, we recommend to start mitotane therapy as soon as clinically possible (+OOO).
We recommend monitoring of blood concentration of mitotane. The general aim is to reach a mitotane blood level above 14 mg/L (+OOO).
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