Long-Term Follow Up and Treatment Outcomes of A 2 Year-Old-Boy With Metastatic Testosterone-Secreting Adrenocortical Carcinoma

Abstract

Objective: Treatment of metastatic adrenocortical carcinoma is challenging and long-term survival rates are exceedingly low. Long-term outcome data for pediatric patients who received mitotane is very limited. Methods: We describe the case of a two-year-old boy with adrenocortical carcinoma with a lung metastasis. He was treated with surgery, chemotherapy and mitotane, and remains disease-free 13 years after diagnosis. Results: Key endocrine issues learned from this case: Adrenal-derived sex-steroid and IGF-2 levels are correlated with disease status; very high doses of glucocorticoid and mineralocorticoid are required while on treatment of mitotane; and central precocious puberty needs to be detected and treated in a timely manner to preserve final adult height. Conclusion: We report a case of pediatric adrenocortical carcinoma with metastasis that was successfully treated with surgery, chemotherapy and adjuvant therapy with mitotane. Appropriate endocrine testing and management are important for long-term survival and quality of life.