The international surgical journal with global reach

This is the Scientific Surgery Archive, which contains all randomized clinical trials in surgery that have been identified by searching the top 50 English language medical journal issues since January 1998. Compiled by Jonothan J. Earnshaw, former Editor-in-Chief, BJS

Diagnosis, treatment and outcome of adrenocortical cancer. BJS 2015; 102: 291-306.

Published: 17th February 2015

Authors: R. Mihai

Background

Adrenocortical cancer (ACC) is a rare disease with a dismal prognosis. The majority of patients are diagnosed with advanced disease and raise difficult management challenges.

Method

All references identified in PubMed, published between 2004 and 2014, using the keywords ‘adrenocortical cancer’ or ‘adrenal surgery’ or both, were uploaded into a database. The database was interrogated using keywords specific for each field studied.

Results

In all, 2049 publications were identified. There is ongoing debate about the feasibility and oncological outcomes of laparoscopic adrenalectomy for small ACCs, and data derived from institutional case series have failed to provide an evidence level above expert opinion. The use of mitotane (1‐(2‐chlorophenyl)‐1‐(4‐chlorophenyl)‐2,2‐dichloroethane) in combination with chemotherapy in the treatment of metastatic disease has been assessed in an international randomized trial (FIRM‐ACT trial) involving patients with ACC. Based on this trial, mitotane plus etoposide, doxorubicin and cisplatin is now the established first‐line cytotoxic therapy owing to a higher response rate and longer median progression‐free survival than achieved with streptozocin–mitotane. For patients with tumours smaller than 5 cm and with no signs of lymph node or distant metastases, survival is favourable with a median exceeding 10 years. However, the overall 5‐year survival rate for all patients with ACC is only 30 per cent.

Conclusion

Open and potentially laparoscopic adrenalectomy for selected patients is the main treatment for non‐metastatic ACC, but the overall 5‐year survival rate remains low.

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