Management and prognosis of adrenocortical tumors in children: can we find out an appropriate points-scoring system to predict prognosis?

Abstract

Adrenocortical tumors (ACTs) are rare in childhood. There are no recognized criteria to exactly distinguish between benign and malignant forms, or predict prognosis. The incidence of tumor varies across geographic regions or ethnicities, as well as malignant proportion. The aim of this study is to examine a single institution's experience with pediatric ACTs and to validate the prognostic value of the biologic/pathologic criteria of Wienecke. Records of 26 pediatric ACTs between 1994 and 2016 in our center were reviewed retrospectively. The data recorded of each patient included clinical characteristics, treatment, pathologic findings, disease stating, and outcome. Tumors were categorized according to the Wienecke criteria. All patients underwent primary surgical excision, including negative margins in 20 cases. Stage distribution at diagnosis was: ST I 12, ST II 8, ST III 5, and ST IV 1. According to Wienecke scoring system, 13 cases were <3 criteria, 6 cases were =3 criteria, and 7 cases were >3 criteria. At median follow-up of 34.5 months, 18 patients survived without evidence of disease and 8 patients had lethal outcome. There was a strong association between high Wienecke score and both high stage and adverse outcome. Wienecke criteria can be an appropriate points-scoring system to predict prognosis for adrenocortical tumors in children. Complete surgical resection with negative margins is optimal for survival.