Complete response of metastasized adrenal cortical carcinoma with o,p'-DDD. Case report and literature review

This study concerns the history of a male patient with hormone-producing adrenal cortical carcinoma. Six months after resection of the primary tumor, lymph node metastases were detected and treatment with o,p'-DDD[1,1 dichloro-2-(o-chlorophenyl)-2-(p-chlorophenyl)ethane] (Lysodren/mitotane) was started. After 2 years, a complete histologically proven response was confirmed at laparotomy and is still sustained 2 years after the discontinuation of treatment. As a consequence of insufficient steroid replacement the patient suffered bouts of adrenal insufficiency. After 1 year of treatment, the measurement of the plasma levels of o,p'-DDD showed an accumulation of the drug. At that time, progressive major central nervous system toxicity occurred, which proved to be reversible on discontinuation of the treatment.