Endocrine Abstracts

Case presentation: We present a case of a 32-year-old male who presented with Cushing’s syndrome. Investigations confirmed a diagnosis of metastatic adrenal cortical carcinoma (ACC) with bilobar liver and multiple lung metastases. He underwent primary tumour resection (histology confirmed 21 cm ACC, T2N0M1, ENSAT stage IV, ki-67 22%), and he commenced mitotane and etoposide, doxorubicin and cisplatin (EDP) chemotherapy. There was a good partial response (PR) with reduction in the size of liver lesions and complete resolution of all lung metastases. Liver only disease was targeted by left segment liver resection, followed by selective internal radiation therapy (SIRT). This was well tolerated and resulted in initial complete response (CR). On subsequent imaging there were two small areas in segment 7 and 8 with viability, and a new right upper lobe lung nodule. He underwent VATS lobectomy for the lung lesion (histologically confirmed metastasis) and repeat SIRT to the segment 7 lesion and irreversible electroporation (IRE) for the segment 8 lesion. Post-treatment imaging revealed a good response, but showed an ablation-related segmental portal vein thrombus. Clinically he is well and is on therapeutic dose anticoagulation. A 6-month restaging scan post repeat SIRT/IRE demonstrated no measurable viable disease.

Discussion: Metastatic ACC has a poor prognosis with limited treatment options. This case highlights the successful use of a multimodality strategy for disseminated metastatic ACC, with combination systemic and locoregional therapies including SIRT to achieve complete treatment response. To our knowledge there are only five published case reports of SIRT in patients with ACC. Two patients had CR and 3 had PR. The reported progression-free survival (PFS) ranged 1-3 years, with minimal adverse events reported.

Conclusion: SIRT has been used effectively to manage metastatic ACC with liver only disease. Prospective studies are warranted to investigate its role in treatment of metastatic ACC.