I. Bergada et al., FUNCTIONAL ADRENAL-CORTICAL TUMORS IN PEDIATRIC-PATIENTS - A CLINICOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL STUDY OF A LONG-TERM FOLLOW-UP SERIES, Cancer, 77(4), 1996, pp. 771-777
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.