[Adrenocortical carcinoma in children: retrospective study of 54 cases].

Background: Adrenal tumors rarely occur in childhood. Their criteria for malignancy, as well as the effects of chemotherapy remain poorly defined.

Population: Fourty-five children (median age: 4 years) with an adrenal tumor diagnosed between 1973 and 1993 were included in this study.

Results: Seventy-six percent of the children showed various degrees of virilization. Tumor was palpable in 57%. Most patients (80%) had local disease, 7% loco-regional disease and 13% distant metastases. Forty-five children underwent an apparently complete surgical resection. Recurrence occurred 2 to 17 months after surgery in 18 of them (40%). Twenty-four children received medical treatment (o.p'-DDD or chemotherapy) and one-third had a tumoral response. The overall 5 year survival rate was 49%.

Conclusions: Adrenocortical neoplasms have a poor prognosis in childhood. Complete resection is the only effective and potentially curative treatment. Currently no effective chemotherapy exists, and the value of adjuvant therapy remains unproven. Multicentric studies are underway to evaluate the efficacy of therapeutic approaches.