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

Abstract

Background Adrenocortical carcinoma is a rare but aggressive form of endocrine neoplasm that confers a poor prognosis. To date, the only Australian data published is from New South Wales. This paper describes our experience in Western Australia with a focus on surgical approach and outcomes. Methods A retrospective study of patients treated for adrenocortical carcinoma in Western Australia over 14 years was performed. Results Over the 14‐year period, a total of 33 patients underwent surgery for adrenocortical carcinoma. Resection outcomes were superior in an open en bloc approach with an 85% R0 margin (P = 0.007). Local recurrence rates were lowest in an open en bloc approach (11%) compared to laparoscopic (75%). Multivariate analysis showed that an en bloc resection is highly correlated with an R0 resection (P < 0.05) and significantly associated with lower (odds ratio = 0.06) local recurrence (P = 0.009). Higher volume surgeons (>5 cases) had lower operative times and blood loss. Compliance with mitotane was significantly improved with close monitoring of levels. The European Network for the Study of Adrenal Tumours (ENSAT) stage at presentation was most predictive of long‐term survival with 100% of stage I patients alive compared to 53% of stage II, 25% of stage III and 17% of stage IV patients at the end of the follow‐up period. Conclusion An open en bloc approach with a low threshold for multi‐visceral resection performed by high volume surgeons have improved outcomes in local recurrence, operative times and blood loss.

Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

“…Aldosterone secreting ACC is uncommon and accounted for approximately 8% of cases. In addition, mixed hormone production was seen in 15% to 25% of functioning ACCs [ 15 , 22 ].…”

Non-functioning adrenocortical carcinoma requiring radical nephrectomy-case report with review of literature

“…Aldosterone secreting ACC are accounted for 8% of cases and mixed hormone production in15% to 25% of functioning ACCs. 16 Non-functioning ACCs commonly present with abdominal mass, abdominal pain and other constitutional symptoms of malignancy. They can also present with paraneoplastic symptoms like hypoglycaemia (secondary to insulin growth factor 2 [IGF2] production) and clinical manifestations related to ACTH production.…”