Clinical and prognostic aspects of adrenocortical neoplasms in childhood

Background. A retrospective study of 54 children was undertaken to define t he clinical presentation and secretory patterns of adrenal tumors and to ev aluate the outcome of surgical resection and medical therapy. Procedures. D ifferent factors were studied in univariate and multivariate analysis by us ing 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 f rom initial symptoms. At initial examination, we found that 76% of children were virilized. Ninety-four percent of the tested tumors secreted androgen s, which were associated with glucocorticoids in 36%. Adrenal tumors in chi ldren were smaller than in adults. Half of them measured less than 10 cm. T here 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. Med. Pediatr. Oncol. 32:106-111, 19 99. (C) 1999 Wiley-Liss, Inc.