PURPOSE

Adrenocortical carcinoma (ACC) is a rare tumor in children with important distinctions from the adult disease. We reviewed the National Cancer Data Base (NCDB) to determine factors associated with long-term survival.

METHODS

The NCDB was queried for patients less than 18 years of age who were diagnosed with ACC between 1998 and 2011. Kaplan-Meier analysis was utilized to determine factors significantly associated with overall survival.

RESULTS

A total of 111 patients were included (median age: 4 years, 69% female). ACC was more common in the youngest cohort, with 48% of cases occurring in children younger than the age of 3. Median tumor size was 9.5 cm (IQR: 6.5-13.0), and 87% of patients underwent some form of surgical resection. Among children with available data, 19 of 62 presented with metastases. Overall 1- and 3-year survival was 70% and 64%, respectively. Age, tumor size, extension of tumor into surrounding tissue, and metastatic disease were all found to be significantly associated with survival. Among patients who underwent a surgical procedure, margin status was also found to be significantly associated with survival.

CONCLUSION

Age, tumor size, extension of tumor, metastatic disease, and margin status are significantly associated with long-term survival in children with adrenocortical carcinoma.