Adrenocortical carcinoma in children and adults: Two decades experience in a single institution.

CONTEXT: Adrenocortical carcinoma (ACC) occurring in children and adults show distinct characteristics. However, due to rarity of the disease no large series addressing this issue has been published.

AIMS: The aim of this study was to study clinico-pathologic profile and outcome of ACC in children and adults.

SETTINGS AND DESIGN: Tertiary referral center. Retrospective study (January 1990-June 2011).

SUBJECTS AND METHODS: Forty-five patients with ACC were included; 16children (aged < 18 years) and 29 adults. Clinical details, hormonal profile, operation records, pathology reports and follow-up findings were noted and compared. Survival analysis was performed using Kaplan-Meier method. Log rank test and Cox regressionan alysis were performed.

RESULTS: Mean age was 8 ± 5.7 (M: F = 1:2.1) in children and 44.4 ± 15 years (M: F = 1:1.1) in adult groups. Prevalence of functioning tumors was significantly high in children (87.5 vs. 31% P = 0.001), while prevalence of incidentalomas was high in adults (6.3 vs. 51.7% P = 0.05). Tumor stage distribution at presentation, mean diameter (10.9 vs. 13.7 cm), and weight (392.9 vs. 892.9 g) didn't differ significantly in two groups. Adults had better albeit non-significant 5 year overall survival (OS) than children (0 vs. 13%). On univariate analysis stage of disease (P = 0.008), surgical intervention (P = 0.004), Weiss score (P = 0.04) and hormonal secretion (P = 0.04) were significantly associated with OS in adults but not in children. No factor was found significant on multivariate analysis.

CONCLUSIONS: Except for high prevalence of functioning tumors in children, clinico-pathologic attributes and outcome of ACC in the two groups didn't differ significantly.