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CORTICAL ADRENAL TUMORS IN INFANCY: PROGNOSTIC VALUE OF PCNA/CYCLIN VERSUS TUMOR WEIGHT

Controversy exists to determine which variable is a reliable predictor of clinical outcome of adrecortical tumors in children. We analyzed retrospectively 17 patients with functional adrenal tumors, and assessed their clinical outcome by comparing tumor weight, classical histologic features and percentage of proliferating cell nuclear antigen (PCNA)/cyclin in their tumor cells in order to delineate which variable correlates best with clinical outcome. Ten patients had Cushing Syndrome and 7 a pure Virilizing Syndrome. Mean age at diagnosis was 5.5±4.3 years(range 0.4-14). Thirteen patients with a benign course have been followed for 10.1±4.3 years(range 3.5-18). All these patients but one(185 gr) had a tumor weight of less than 100 gr(mean 60.8±52 gr). Two patients had a malignant course, one died 5 months after surgery with metastasis and the other presented lung metastasis 18 months after surgery. Their tumor weights were 1000 and 780 gr respectively. All the histology parameters such as cellular pleomorfism, neocrosis, calcifications, capsular and vascular invasion, were randomly found in all patients and did no correlate with clinical outcome. In the former 13 patients staining with PCNA/cyclin was found in 16±25% of the tumor cells(median=15, range=0 to 90%); the two who had malignant course had values of 60 and 70%. We found a positive correlation between tumor weight and PCNA/cyclin(r=0.60, p=0.015). Our data shows that PCNA/cyclin could be a further aid to predict clinical behavior on these infrequent tumors althought the occasional presence of small tumors with high PCNA/cyclin values, indicates that this parameter should not be used alone.