[Adrenal cortex carcinoma in childhood and adolescence]

The presented paper includes the medical records of two girls with adrenal cortical carcinomas on the one hand and the detailed analysis of a collected series of 150 equal cases in pediatric literature on the other hand. In our patients there were a primarily metastatic, non-functioning tumour and a locally invasive, hormone-secreting neoplasm respectively. The latter one produced a Cushing's syndrome with signs of virilization. In this case surgical removal was followed by postoperative irradiation and adjunctive cytostatic therapy with cyclophosphamide and adriamycin for one year. But tumour recurrence occurred within 6 months. A treatment with the antitumour agents aminogluthetimide and o,p' DDD was transiently effective. However drug-induced side effects necessitated the stop of this regimen after 5 months. The analysis of 150 case reports revealed a peak between 1 and 3 years of age, a striking prevalence of the female sex in all age groups, and a metastasizing rate of about 30% at the time of diagnosis. Regional structures, liver and lung were the main locations of metastatic lesions. The occurrence of second primary neoplasms and the association with a group of other conditions particularly congenital disorders have proved to be a remarkable feature of the disease. In primary adrenal cortical tumours the determination of the neoplasm's biological behaviour by morphologic criteria alone can often be very problematical. This fact is especially stressed.