We present an evolving 21‐year management experience of adrenocortical carcinoma at an Australian specialist endocrine surgical unit, with a perspective towards the approaches, outcomes and lessons learnt. Disease stage, R0 resection and Ki‐67 level are key discriminators for outcomes. We found that it is possible to have relatively long survival of Stage 4 disease, with aggressive management of oligometastatic disease.
Background
Adrenocortical carcinoma is a rare and heterogeneous malignancy with poor outcomes. Recent research has suggested that outcomes may be improved by centralization of care in specialist centres. We review our evolving 21‐year experience in managing adrenocortical carcinoma with a view towards outcomes and lessons learnt.
Methods
A retrospective study of patients treated in our specialist endocrine surgical unit over 21 years was undertaken.
Results
Thirty‐five patients were treated from diagnosis, 29 forming a primary study cohort. Additionally, seven patients were referred to us for quaternary care, forming a secondary study cohort. The European Network for the Study of Adrenal Tumours (ENSAT) stage and immunohistochemical marker Ki‐67 index were strong prognostic indicators for survival.
Conclusions
Early stage, complete resection and Ki‐67 <10% are the best prognosticators for survival. Aggressive surgical resection at index operation and of recurrent oligometastatic disease along with multimodal adjuvant treatment has led to long‐term survivors of patients with Stage 4 disease in our aggregate cohort.
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