Adrenocortical carcinoma
ICD-10 C74.0 · ICD-11 2D11.0

Treatment of ENSAT Stage I–III Adrenocortical Carcinoma Amenable to Complete Resection

This protocol covers patients with adrenocortical carcinoma (ACC) staged at ENSAT I, II, or III for whom complete (radical) en bloc surgical resection is achievable — defining the patient population and the adjuvant strategy that follows.

Clinical Scenario

Staging is based on the European Network for the Study of Adrenal Tumours (ENSAT) classification system. Patients with ENSAT stage I and stage II disease — and the majority of those with stage III — are candidates for radical resection, which forms the cornerstone of curative intent in this setting.

Adjuvant Treatment Approach

After complete surgical resection, adjuvant mitotane therapy is initiated as early as clinically feasible — particularly in patients assessed to carry a higher risk of recurrence. Glucocorticoid replacement is co-administered throughout mitotane treatment. Radiation therapy directed at the prior tumour site may be incorporated in patients with specific resection margin status or stage III disease. In carefully selected patients with very high recurrence risk, additional cytotoxic therapy may be considered on an individualised basis.

Full sequencing, selection criteria, individualised decision points, and treatment duration are contained in the complete protocol — not shown here.

Monitoring Goal

Mitotane blood levels are monitored throughout treatment. The therapeutic target is a blood level of 14–20 mg/L, with the general aim of exceeding 14 mg/L.

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References

DOI: 10.1530/EJE-18-0608

At initial diagnosis, we recommend using the European Network for the Study of Adrenal Tumours (ENSAT) staging classification (+++O).

All patients with stage I + II and most patients with stage III should be amenable to radical resection.

We 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%).

We recommend glucocorticoid replacement in all patients treated with mitotane (except those with ongoing cortisol excess).

The panel suggests considering radiation in addition to mitotane therapy on an individualized basis in patients with R1 or Rx resection or in stage III.

However, the panel suggests considering adjuvant chemotherapy in selected patients with very high risk for recurrence.

We recommend monitoring of blood concentration of mitotane. The general aim is to reach a mitotane blood level above 14 mg/L (+OOO).

Plus oral mitotane aiming at a blood level between 14 and 20 mg/L.

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