Clinicopathological Features of Pediatric Functional Adrenocortical Carcinoma Diagnosed by Weiss Criteria; An Analysis of Four Cases.

Abstract

Histological differentiation between benign and malignant adrenocortical tumors is currently based on the criteria of Weiss et al. (Weiss criteria) and vimentin immunostaining, but the prognostic values of the criteria in pediatric cases are still unclear. We analyzed the histological features, immunostaining status of p53, vimentin, Ki67 and cyclin-A, and clinical outcomes of four pediatric adrenocortical carcinomas diagnosed based on the Weiss criteria. One child developed metastases and recurrence, and died of the disease six months after tumor resection. The other three were disease free two to nine years after surgery. Tumor necrosis, and vascular and capsular invasion, three items of the Weiss criteria, and large tumor size seemed to be correlated with worse prognosis. On the other hand, mitotic count and atypical mitosis, two major factors of the Weiss criteria, and tumor proliferating fraction (TPF), as well as the immunostaining status of vimentin, seemed irrelevant to the clinical outcomes. In conclusion, some pediatric adrenocortical carcinomas diagnosed according to the Weiss criteria may behave benignly, and diagnostic criteria for pediatric adrenocortical tumors remain to be defined.