Disease-free remission of metastatic adrenocortical carcinoma following en bloc tumor resection and neoadjuvant chemotherapy for suspected adrenohepatic infiltration

Abstract

A 3 year old boy with a family history of Li-fraumeni Syndrome presented with a right-sided adrenocortical carcinoma, hepatic, and pulmonary metastases. He underwent eight cycles of chemotherapy with Cisplatin, Etoposide, Doxorubicin and Mitotane. Follow-up CT Thorax/Abdomen/Pelvis with contrast demonstrated a reduction in size of the right suprarenal mass, decreased attenuation of liver metastases and one remaining pulmonary nodule. Discussion at multidisciplinary meeting ensued and open adrenalectomy and resection of hepatic metastases was recommended. Intra-operatively, gross appearance of adrenohepatic infiltration into the right hepatic lobe was identified and the decision was made to perform complete, en bloc resection of mass. The remaining pulmonary nodule resolved radiologically. The patient remains well and continues on maintenance adrenolytic therapy with mitotane. Thirteen months post-operatively, no locoregional disease recurrence has been identified on imaging. Disease-free remission is a rare occurrence in metastatic adrenocortical carcinoma. To the author's knowledge this is the first case report demonstrating the detailed surgical management of a child with an initial presentation of metastatic adrenocortical carcinoma.

Keywords

• Adrenocortical carcinoma
• Li-fraumeni syndrome
• En bloc resection