Predictors of survival in pediatric adrenocortical carcinoma: a Surveillance, Epidemiology, and End Results (SEER) program study.

Background: Adrenocortical carcinoma (ACC) is rarely described in children. There is variation in incidence worldwide. This study sought to identify national incidence rates and independent prognostic indicators for children.

Methods: The SEER database was queried for the years 1973 through 2008 for all patients with ACC less than 20 years of age. Incidence rates and survival were analyzed accounting for clinical and demographic factors. Cox proportional-hazards regression was used to identify factors associated with disease-specific survival.

Results: Eighty-five patients (57 F: 28 M) were identified. Annual ACC incidence was 0.21 per million. Young patients (≤ 4 years) were noted to have more favorable features than older patients (5-19 years) and more likely to have local disease (76% vs. 31%, p < 0.001), tumor size < 10 cm (69% vs. 31%, p = 0.007), and better 5-year survival (91.1% vs. 29.8%, p < 0.001). After adjustment, the most significant predictors of cancer-specific death were age 5-19 years (HR 8.6, p = 0.001) and distant disease (HR 3.3, p = 0.01). After accounting for tumor size, only age maintained statistical significance (HR 9.9, p = 0.009).

Conclusions: Our study represents one of the largest reviews of pediatric ACC. An age of ≤ 4 years was associated with better outcome. Potential factors responsible for this include patient and tumor related factors.