Background:
A retrospective study of 54 children was undertaken to define the clinical presentation and secretory patterns of adrenal tumors and to evaluate the outcome of surgical resection and medical therapy.
Procedures:
Different factors were studied in univariate and multivariate analysis by using the cox proportional hazard model.
Results:
Median age at diagnosis was 4 years. boys and girls were affected equally. the disease was revealed by virilization (61%) or by a palpable mass (39%) with a 0.1-5.5 year delay from initial symptoms. at initial examination, we found that 76% of children were virilized. ninety-four percent of the tested tumors secreted androgens, which were associated with glucocorticoids in 36%. adrenal tumors in children were smaller than in adults. half of them measured less than 10 cm. there were recurrences in 40% of children. the survival rate at 5 years was 49%, 70% if resection was microscopically complete and 7% if not (p < 0.001).
Conclusions:
In children, rare adrenal tumors have different diagnostic and prognostic characteristics than in adults; however, recurrences remain frequent. the efficacy of chemotherapy, mainly o,p'-ddd (mitotane), remains to be evaluated in comparative trials.