Correlation of pathologic features with clinical outcome in pediatric adrenocortical neoplasia. A study of a Brazilian population. Brazilian Group for Treatment of Childhood Adrenocortical Tumors.

Although the relationship between pathologic features and clinical outcome is well established in adult adrenocortical neoplasms, the prognostic value of these features in pediatric adrenocortical neoplasms (PACN) is unclear. In a series of PACNs from 54 Brazilian children, the authors retrospectively investigated the prognostic value of histologic classification, ploidy, proliferative index, and size (as tumor weight or greater diameter). Histologic classification was most predictive of clinical behavior: there were no failures in 11 adenomas, 5 failures in 27 low-grade carcinomas, and 9 failures in 16 high-grade carcinomas (P = .0003). Tumor weight was predictive of failure in tumors weighing > or = 100 versus < 100 g (P = .04), and a trend was found toward failure among tumors measuring > or = 5 cm, as opposed to those < 5 cm (P = .07). Proliferative index was marginally related to failure (P = .05 at < 11% vs. > or = 11% and .07 at < 10% vs. > or = 10%), and ploidy was not significantly predictive of outcome (P = .25). Histologic type and tumor weight were the most reliable predictors of outcome in PACN.