Functioning and nonfunctioning adrenocortical carcinoma: clinical presentation and therapeutic strategies

Abstract

Adrenocortical cancers are relatively rare endocrine tumors that usually present when hormonally active or after they have become large and metastasis has occurred. Consequently, the 5-year survival rate is 20% to 35%. Surgical removal remains the only form of therapy proven to prolong survival. Mitotane is the most accepted form of chemotherapy. For the approximately 20% to 25% of patients whose tumors respond to mitotane, survival is prolonged.