ADRENOCORTICAL NEOPLASMS IN CHILDHOOD - A RETROSPECTIVE STUDY OF 54 CASES

Background.-Adrenal tumors rarely occur in childhood. Their criteria f or malignancy, as well as the effects of chemotherapy remain poorly de fined. Population.-Fourty-five children (median age: 4 years) with an adrenal tumor diagnosed between 1973 and 1993 were included in this st udy. Results.-Seventy-six percent of the children showed various degre es of virilization. Tumor was palpable in 57%. Most patients (80%) had local disease, 75 loco-regional disease and 13% distant metastases. F orty-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 chemoth erapy) and one-third had a tumoral response. The overall 5 year surviv al rate was 49%. Conclusions.-Adrenocortical neoplasms have a poor pro gnosis in childhood. Complete resection is the only effective and pote ntially curative treatment. Currently no effective chemotherapy exists , and the value of adjuvant therapy remains unproven. Multicentric stu dies are underway to evaluate the efficacy of therapeutic approaches.