FUNCTIONAL ADRENAL-CORTICAL TUMORS IN CHILDHOOD - A STUDY OF PLOIDY, P53-PROTEIN AND NUCLEOLAR ORGANIZER REGIONS (AGNORS) AS PROGNOSTIC MARKERS

Prognostic markers in pediatric adrenal cortical tumors are difficult to define. We determined the ploidy, immunostaining of p53-protein and number of nucleolar organizer regions (AgNORs) in 16 such tumors and related them to clinical outcome, tumor weight (TW) and histologic Wei ss' criteria. Eleven females and 5 males aged 0.4 to 15.6 years were f ollowed for 8.7 years; 10 presented Gushing's and 6 virilization syndr ome. Diploid (n=4, x TW=269 g, range: 17-800 g) and near-diploid tumor s (n=3, x TW=55 g, range: 20-85 g) had good outcome, Weiss' criteria w ere 0-7, and p53 reactivity was negative in all. Among the aneuploid t umors (n=9, x TW=298 g, range: 7-1000 g), 6 had good outcome, 2 presen ted metastasis and 1 was lost to follow-up; Weiss' criteria were 2-8 a nd p53 reactivity was positive in 3 tumors (2 of them of malignant evo lution). AgNORs number was not different in cases of good or poor outc ome (3.65+/-1.9 vs 2.83+/-1.1). Our findings indicate that diploid and near-diploid cases had always a good outcome regardless of tumor weig ht. In aneuploid cases, tumor weights < 100 g had good outcome, while those > 750 g had poor prognosis. Malignant tumors were aneuploid and had reactivity to p53-protein. Good outcome in aneuploid tumors < 100 g is probably due to early treatment. The expression of p53-protein ap pears as a promising marker of poor prognosis. Weiss' criteria and AgN ORs were not useful in the present series.