Adrenocortical Carcinoma Clinical Practice Guidelines (2018)

European Society of Endocrinology

Reviewed and summarized by Medscape editors

November 05, 2018

Clinical practice guidelines for the management of adrenocortical carcinoma in adults were released in October 2018 by the European Society of Endocrinology.[1]


Perform a detailed hormonal workup of all patients with suspected adrenocortical carcinoma (ACC) to identify potential autonomous excess of glucocorticoids, sex hormones, mineralocorticoids, and adrenocortical steroid hormone precursors.

Perform a chest CT in addition to an abdominal-pelvic cross-sectional imaging (CT or MRI) in any case where there is high suspicion for ACC.

Do not use adrenal biopsy in the diagnostic workup of patients with suspected ACC unless there is evidence of metastatic disease that precludes surgery.


Complete en bloc resection of all adrenal tumors suspected to be ACC is recommended. Enucleation and partial adrenal resection is not recommended.

Open surgery for all tumors with radiological findings suspicious of malignancy and evidence for local invasion is recommended.

Routine loco-regional lymphadenectomy should be performed with adrenalectomy for highly suspected or proven ACC. At a minimum, it should include the periadrenal and renal hilum nodes. All suspicious or enlarged lymph nodes identified on preoperative imaging or intraoperatively should be removed.

Perioperative hydrocortisone replacement in all patients with hypercortisolism that undergo surgery for ACC is recommended.

Pathological Work-up

Use immunohistochemistry for steroidogenic factor-1 (SF1) for the distinction of primary adrenocortical tumors and non-adrenocortical tumors.

Use the Weiss system for the distinction of benign and malignant adrenocortical tumors.

Use Ki67 immunohistochemistry for every resection specimen of an adrenocortical tumor.

Adjuvant Therapy

Adjuvant therapy is not recommended for adrenal tumors with uncertain malignant potential.

Adjuvant mitotane treatment is recommended for those patients without macroscopic residual tumor after surgery who have a high risk of recurrence.

Administer adjuvant mitotane for at least 2 years, but not longer than 5 years, in patients without recurrence.

Treatment of Recurrent and/or Advanced ACC

For advanced ACC not amenable to complete surgical resection, local therapeutic measures (eg, radiation therapy, radiofrequency ablation, chemoembolization) are of particular value.

Routine use of adrenal surgery in case of widespread metastatic disease at the time of first diagnosis is not recommended.

In patients with advanced ACC at the time of diagnosis not qualifying for local treatment, either mitotane monotherapy or mitotane + EDP is recommended.

Surgery is recommended for patients with recurrent disease and a disease-free interval of at least 12 months.

Pregnancy and ACC

Prompt surgical resection is recommended when an adrenal mass suspected to be an ACC is diagnosed in a pregnant patient.

Patients should avoid pregnancy while on mitotane treatment.


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