Treatment and management of adrenal cancer in a specialized Australian endocrine surgical unit: approaches, outcomes and lessons learnt

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.

Management of adrenocortical carcinoma in Western Australia: a perspective over 14 years

“…Hormonal hypersecretion is described in up to 75% of ACCs 3 . Cortisol hypersecretion is most common, ranging from 23% to 50%, followed by DHEAS 9–19%, a combination and rarely aldosterone secreting only 10,19,20 . A total of 65% of patients in our series presented with hypercortisolism, 25% with androgen excess and 12% a combination of both.…”

“…3,8,9 R1 and R2 resection margins are associated with higher local recurrence rates with reported rates between 36% and 85%. [10][11][12] Our current surgical practice is to perform an open en bloc approach with two consultants present when possible for suspected ACC cases with a very low threshold for multivisceral resection. The presence of two consultants increases operative experience in these rare tumours.…”

“…Nonetheless, there is little debate that achieving an R0 resection confers the best outcome in terms of recurrence and survival. To date, the only Australian data published is from New South Wales (NSW) 9,10 . This paper describes our experience in Western Australia, focusing on surgical approach and outcomes.…”

Summary of clinical characteristics of common primary adrenal malignancies in adults

“…The clinical results of adjuvant therapy have been disappointing. Some indicated adjuvant radiotherapy may prolong the time to ACC local recurrence [35,36] , some even thought adjuvant therapy was associated with decreased recurrence [24] , others suggested it was of bene t in stage IV disease [32,37] . However, there was a consensus that it was no bene t for OS [24,35,38] .…”

“…Furthermore, based on advancing research, pathological markers such as the Ki67 index were also important prognostic indicators for ACC [37,40,55] . What's more interesting was that the neutrophil-to-lymphocyte ratio (NLR) and lymphocyte-to-monocyte ratio (LMR) might be new biomarkers for predicting the prognosis of adrenal tumor patients.…”