Resection of Adrenocortical Carcinoma with Bilobar Liver Metastasis Following Neoadjuvant Therapy Using a Modified Mini-ALPPS Approach: A Case Report

Abstract

Introduction: Adrenocortical carcinoma (ACC) is an aggressive tumor with high proportion of patients presenting with metastatic disease, most commonly in the liver. Prognosis in this population is extremely poor. Resection of the primary tumor and liver metastasis offers a survival benefit in well-selected patients. However, the extent of surgery is often significant and can limit the ability to accomplish a safe marginnegative resection. Presentation of case: A 35-year-old male presented with a large left ACC (15.2cm) and multiple bilobar liver metastases (1.5-12.5cm). He was treated with mitotane and chemotherapy / immunotherapy, with excellent response. Multidisciplinary discussion led to recommendations for a curative-intent approach with surgery. A staged approach was performed for the resection, using a modified Mini-ALPPS technique. A complete margin-negative resection of all disease was accomplished. The patient recovered well and remains free of disease 24-months following diagnosis. Discussion: This case highlights novel components of treatment for metastatic ACC and for hepatectomy for bilobar liver metastasis. The decisions to proceed to surgery for complete resection and to use a staged approach with a modified Mini-ALPPS technique were both critical components to render the patient disease-free. Appropriate expertise and multidisciplinary teamwork are essential for implementation of these approaches. Conclusion: Neoadjuvant chemotherapy for stage IV ACC can result in disease control and improved selection of candidates for curative-intent surgery. In the setting of bilobar liver disease and a large primary in place, a modified Mini-ALPPS approach provides a safe and feasible way to accomplish complete resection and improved survival.