Childhood adrenocortical tumors: case series and reevaluation of prognosis--a 24-year experience.

ABSTRACT

Adrenocortical neoplasms are rare in childhood and adolescence. The prognostic significance of tumor size, weight, and histological grade are 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 identified. Six presented with virilizing symptoms, two with cushingoid symptoms, one with both, and two others had nonspecific symptoms. The interval between onset of symptoms and diagnosis was an average of 18 months (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 intraoperative spillage. Median tumor weight was 94.5 g (range, 4 to 750 g). Three lesions were less than 5 cm in maximal width, six were between 5 and 10 cm, and two were greater than 10 cm. Two tumors had capsular or vascular invasion. Three patients received chemotherapy one who had inoperable metastatic disease, and two based on clinical and histopathologic findings. Ten patients are doing well, without evidence of recurrent 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. The medically treated patient who had metastatic disease died 3 years after diagnosis. A review of the pediatric literature, in some cases, indicates that larger tumors have a worse prognosis, while other investigators claim histological grade is more important. The authors' results do not support these conclusions, but rather suggest that in the pediatric population, when excision is complete, guarded optimism is warranted even with tumors larger than 5 cm. Addendum Since submission of the manuscript, patient 4 has been operated on twice for local recurrences 13 and 16 months after the initial surgery. She was the only patient in the series to have an intraoperative capsular tear. All other surgical patients remain free of disease.