Grace T Y Kwok1, Jing-Ting Zhao1, Anthony R Glover1,2,3, Julian C Y Ip1,3, Mark Sywak2,3, Roderick Clifton-Bligh1,2,4, Stephen Clarke1,5, Bruce Robinson1,2,4, Stan B Sidhu1,2,3. 1. Cancer Genetics Laboratory, Kolling Institute, Northern Sydney Local Health District, Sydney, New South Wales, Australia. 2. Faculty of Medicine and Health, Royal North Shore Hospital, The University of Sydney, Sydney, New South Wales, Australia. 3. University of Sydney Endocrine Surgery Unit, Royal North Shore Hospital, Sydney, New South Wales, Australia. 4. Department of Endocrinology, Royal North Shore Hospital, The University of Sydney, Sydney, New South Wales, Australia. 5. Department of Medical Oncology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
Abstract
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.
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.