Adrenocortical tumors in children

Background/Purpose: Etiopathogenesis and management of pediatric adrenocort ical tumors (ACTs) is still obscure because of the limited number of cases. The aim of this study is to present a clear picture of the entire spectrum of pediatric ACTs by reviewing one of the largest noncollected pediatric s eries treated in a single medical center. Methods: Records of children treated for ACTs in our unit between 1970 and 1999, inclusive, were reviewed. Information recorded for each patient inclu ded age, sex, clinical characteristics, diagnostic methods, stage of diseas e, treatment, pathologic findings, and outcome. The patients were subdivide d into 2 groups: group I, patients with adrenocortical carcinoma (ACC) and group II, patients with adrenocortical adenoma (ACA). These groups were ana lyzed with regard to parameters mentioned above. Results: There were 30 children treated for ACTs in the study period with a mean age of 6.7 +/- 4.2 years (range, 2.5 to 13 years). Of these, 20 had A CC, and 10 had ACA. The tumors were right sided in 22 patients, left sided in 6 and bilateral in 2. Analysis of each group with regard to age and site of tumor showed no significant difference. Endocrine dysfunction was noted in 83% of the patients and virilization was the most common presentation f ollowed by Gushing's syndrome. The most striking difference between 2 group s was the prepondarance of virilization in group II and Gushing's syndrome in group I. In the latter, 14 patients presented with palpable abdominal ma ss and 3 patients with distant metastases. The mean time from initial sympt oms to diagnosis was 8.1 +/- 0.2 months, and this interval was similar in 2 groups, in functional and nonfunctional tumors, and in both sexes. Ultraso und scan, computerized tomography, magnetic resonance imaging, intravenous pyelography, and angiography were used for the diagnosis. All patients with AGA had localized disease, whereas 80% of the patients with ACC had region al or metastatic disease. Total excision was done in all patients with ACA, but only in 13 patients with ACCs. Of the latter, 2 patients underwent ips ilateral nephrectomy, and 1 patient had right hepatic lobectomy plus nephre ctomy. Adjuvant chemotherapy consisting of mitotane (n = 12), mitotane plus cisplatin and etoposide (n = 2) was commenced. Seven patients with ACC had distant metastases postoperatively. The presence of regional disease at pr esentation was associated with a significantly shorter disease-free interva l. All patients presenting with nonfunctional ACC (n = 4), functional ACC t hat have been totally resected (n = 4), and partially resected (n = 3) died of disease within the first 2.5 years after diagnosis. There was no signif icant difference between the functional and nonfunctional ACCs with regard to survival rate. All patients who had distant metastases postoperatively a nd who had partial excision died. Of the surviving 9 patients with ACG. the re are 6 known longterm survivors who are still alive. Conclusions: ACAs are treated by total excision satisfactorily without any complication. For the time being, the most important aspect of therapy for ACCs is early diagnosis and total excision. Partial excision and advanced-s tage disease are the major determinants of poor outcome. None of the clinic al, laboratory, or pathologic features are reliable predictors for recurren ce and discrimination of malignancy in ACTs. Because of the steadily increa sing incidence of precancerous genetic syndromes of adrenal glands and poor prognosis of ACCs, childhood patients of endocrine disorders should receiv e a detailed and vigorous diagnostic evaluation and appropriate treatment a s given to adults. Patients with ACTs should be entered into multi-institut ional trials to adequately assess effective chemotherapy and radiotherapy p rotocols and molecular mechanisms of oncogenesis.