CHILDHOOD ADRENOCORTICAL TUMORS - CASE SERIES AND REEVALUATION OF PROGNOSIS - A 24-YEAR EXPERIENCE

Adrenocortical neoplasms are rare in childhood and adolescence. The pr ognostic significance of tumor size, weight, and histological grade ar e still very much unclear, Eleven patients, (3 boys, 8 girls), with a median presentation age of 7 years (range, 0.8 to 16 years) were ident ified. Six presented with virilizing symptoms, two with cushingoid sym ptoms, one with both, and two others had nonspecific symptoms, The int erval between onset of symptoms and diagnosis was an average of 18 mon ths (median, 8 months). Hormonal profile correlated well with clinical presentation in nine patients. Two patients with nonspecific symptoms had an aldosterone-producing lesion and an androgen-secreting tumor. Ten patients underwent complete surgical excision, with one intraopera tive spillage, Median tumor weight was 94.5 g (range, 4 to 750 g). Thr ee lesions were less than 5 cm in maximal width, six were between 5 an d 10 cm, and two were greater than 10 cm. Two tumors had capsular or v ascular invasion. Three patients received chemotherapy: one who had in operable metastatic disease, and two based on clinical and histopathol ogic findings. Ten patients are doing well, without evidence of recurr ent disease with a median follow-up of 3 years (range, 9 months to 15 years), eight patients have been followed up for more than 2 years, Th e medically treated patient who had metastatic disease died 3 years af ter diagnosis. A review of the pediatric literature, in some cases, in dicates that larger tumors have a worse prognosis, while other investi gators claim histological grade is more important. The authors' result s do not support these conclusions, but rather suggest that in the ped iatric population, when excision is complete, guarded optimism is warr anted even with tumors larger than 5 cm. Addendum: Since submission of the manuscript, patient 4 has been operated on twice for local recurr ences 13 and 16 months after the initial surgery. She was the only pat ient in the series to have an intraoperative capsular tear. All other surgical patients remain free of disease. Copyright (C) 1997 by W.B. S aunders Company.