In collaboration with the European Network for the Study of Adrenal Tumors (ENSAT), the European Society of Endocrinology (ESE) has launched Clinical Practice Guidelines on the Management of Adrenocortical Carcinoma in Adults.
The guidelines address the diagnosis, treatment and follow-up surveillance of patients with adrenocortical carcinoma (ACC), as well as making recommendations on genetic counselling and the management of ACC in pregnancy.
Given the dearth of research on the topic, the guidelines development panel could not reach consensus on a number of issues and it remarked that the evidence for most of the recommendations put forward was weak or very weak.
The ESE recommends adjuvant mitotane treatment for patients without macroscopic residual tumour after surgery and high risk of recurrence. However, it does not recommend for or against adjuvant therapy for patients at low/moderate risk of recurrence (stage I-II, R0 resection and Ki67 ≤ 10%).
The guidelines have been endorsed by the European Society of Endocrine Surgeons, the Endocrine Society, the European Society of Pathology, the American-Australian-Asian Adrenal Alliance (A5), the European Reference Network on Rare Endocrine Conditions (Endo-ERN), and the European Reference Network on Rare Adult Solid Cancers (ERN EURACAN).