FUNCTIONAL ADRENAL-CORTICAL TUMORS IN PEDIATRIC-PATIENTS - A CLINICOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL STUDY OF A LONG-TERM FOLLOW-UP SERIES

BACKGROUND. Controversy exists as to which variable is a reliable pred ictor of clinical outcome of adrenal cortical tumors in children. METH ODS, Twenty patients with adrenal cortical tumors were studied. Tumor weight, histologic features, and percentage of proliferating cell. nuc lear antigen (PCNA/cyclin) in tumor cells were analyzed to determine t he best predictor of clinical outcome. RESULTS, Eleven patients had Gu shing's syndrome with virilization and 9 had virilization without Gush ing's syndrome. The mean age at diagnosis was 7.1 +/- 5.2 years (range , 0.4-15.6 years). Sixteen patients, with good outcomes have been foll owed for 10.7 +/- 7.8 years (range, 3-23 years). All but two patients had a tumor weight of less than 100 g (185 g and 800 g, respectively) (mean 47.7 g +/- 46.4 g). Two patients with large tumors (weighing 100 0 g and 780 g, respectively) had poor outcomes; 1 died 3 months after surgery with metastasis and the other presented with lung metastasis 1 8 months after surgery. Histologic features did not correlate with cli nical outcome. Overall, PCNA stained cells were 6.96 +/- 8.2% (range, 0-32.5%). PCNA values were significantly lower in tumors of patients w ith good outcomes (P < 0.002). Within all tumors, we found a weak corr elation between tumor weight and PCNA (r = 0.51; P < 0.02), but a bett er correlation was found between tumor weight and PCNA in patients wit h Cushing's syndrome (r = 0.70; P < 0.01). Patients with Cushing's syn drome had higher PCNA values than those with virilization syndrome (10 .3 +/- 9.6% vs. 2.8 +/- 3.3%; P < 0.03). CONCLUSIONS, Our data show th at small tumors (less than 100 g) are associated with good outcome; th e two patients with the poorest prognosis had Gushing's syndrome and l arge tumors (more than 100 g). Histologic features are not adequate pr edictors of outcome and PCNA may be useful in tumors of patients with Cushing's syndrome, but this parameter should not be used alone. Two p atients had virilization syndrome, large tumors (185 g and 800 g, resp ectively), and good outcomes, which contradicts with the concept that these tumors are usually associated with poor prognosis. (C) 1996 Amer ican Cancer Society.