Key result
A 2-year-old boy with metastatic adrenocortical carcinoma treated with surgery, chemotherapy, and mitotane remained disease-free 13 years after diagnosis.
Why the study?
Treatment of metastatic adrenocortical carcinoma is challenging with exceedingly low long-term survival rates, and long-term outcome data for pediatric patients receiving mitotane are very limited.
Does surgery, chemotherapy, and mitotane improve long-term survival in a pediatric patient with metastatic adrenocortical carcinoma?
Case Report (n=1)
Does surgery, chemotherapy, and mitotane improve long-term survival in a pediatric patient with metastatic adrenocortical carcinoma?
A pediatric patient with metastatic adrenocortical carcinoma achieved 13-year disease-free survival with surgery, chemotherapy, and mitotane, highlighting the need for careful endocrine management.
Supports multimodal therapy consideration in rare pediatric metastatic adrenocortical carcinoma; leaves open confirmation in prospective studies.
OBJECTIVE: Treatment of metastatic adrenocortical carcinoma (ACC) 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 2-year-old boy with ACC with a lung metastasis. He was treated with surgery, chemotherapy, and mitotane, and remains disease-free 13 years after diagnosis. RESULTS: The key endocrine issues learned from this case include: adrenal-derived sex-steroid and insulin-like growth factor-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 ACC 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.
No takes yet. Share an insight, caveat, or question.
LoGiurato et al. (2020) conducted a case report in Metastatic adrenocortical carcinoma (n=1). Surgery, chemotherapy, and mitotane was evaluated. A 2-year-old boy with metastatic adrenocortical carcinoma treated with surgery, chemotherapy, and mitotane remained disease-free 13 years after diagnosis.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: