Experience with Adrenocortical tumors in children: a report of eleven cases

ABSTRACT

During a period of 15 years from 1975 to 1989, eleven children with adrenocortical tumors were managed with relatively favorable results in our center. Despite its rarity in the pediatric age group, our series is one of the largest reported in the literature in the given period of time. All of our 11 patients presented with hyperfunction of the adrenal cortex. Virilization alone was the most frequent presenting sign, occurring in eight patients. Two patients suffered from Cushing's syndrome and one patient had mixed presentation of Cushing's syndrome and virilization. There were six females and five males in the series. Four of five males presented before two years of age. Female patients presented at an older age. In nine out of 11 patients the tumor was on the right side. Mean age at presentation was 3.55 years. Serum levels of testosterone, dehydroepiandrosterone [DHEA], and cortisol were elevated in all cases tested and apparently are superior alternatives to more traditional measurements of urinary 17- ketosteroids and 17-hydroxy corticosteroids. The majority of our cases were investigated by the employment of IVP and sonography. In one patient I[131] - iodocholesterol scintigraphy was used. Transabdominal approach through a transverse upper abdominal incision is recommended. In this series only one intraoperative complication was encountered. At a mean follow-up of five years, 10 out of 11 children were alive and had no evidence of tumor recurrence. In all patients pre-operative steroid replacement was essential. Contrary to our pathologic reports <adenoma, three carcinoma, and two undetermined>> we found favorable outcomes in the majority of our patients. We conclude that in contradiction to previous concensus, most pediatric adrenocortical tumors will behave as a benign neoplasm and that the size and weight are the only morphologic predictors of their biological behavior