The clinical practice guidelines on adrenocortical carcinoma were released on July 24, 2018, by the ESE.
Recommend that all patients with suspected and proven ACC are discussed in a multidisciplinary expert team meeting.
Recommend that every patient with (suspected) ACC should undergo careful clinical assessment, detailed endocrine work-up to identify autonomous hormone excess, and adrenal-focused imaging.
Recommend that adrenal surgery for (suspected) ACC should be performed only by surgeons experienced in adrenal and oncological surgery aiming at a complete en-bloc resection (including resection of oligo-metastatic disease).
Suggest that all suspected ACC should be reviewed by an expert adrenal pathologist using the Weiss score and providing Ki67 index.
Suggest adjuvant mitotane treatment in patients after radical surgery that have a perceived high risk of recurrence (ENSAT stage III, or R1 resection, or Ki67 >10%). For advanced ACC not amenable to complete surgical resection, local therapeutic measures (eg, radiation therapy, radiofrequency ablation, chemoembolization) are of particular value. However, we suggest against the routine use of adrenal surgery in case of widespread metastatic disease. In these patients we recommend either mitotane monotherapy or mitotane, etoposide, doxorubicin, and cisplatin depending on prognostic parameters. In selected patients with a good response, surgery may be subsequently considered.
In patients with recurrent disease and a disease-free interval of at least 12 months, in whom a complete resection/ablation seems feasible, we recommend surgery or alternatively other local therapies.
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Cite this: Adrenocortical Carcinoma Clinical Practice Guidelines (2018) - Medscape - Aug 09, 2018.