836 DIFFERENTIAL DIAGNOSIS BETWEEN CARCINOMAS AND ADENOMAS OF THE ADRENAL CORTEX IN CHILDREN: A COMPARISION OF 3 HISTOPATHOLOGIC SYSTENS

Abstract

dren treated at our institution for bilateral Wilms tumor without antecedent diagnostic biopsy. All received vincristine, actinomycin D, and doxorubicin for a minimum of 6 weeks prior to surgery. RESULTS: Age at clinical diagnosis ranged from 8 to 63 months. All 5 children had a palpable abdominal mass. Ultrasound failed to detect bilateral disease in 3. All diagnoses were made with cross-sectional imaging. Studies to rule out neuroblastoma were obtained in 4. Time from diagnosis to chemotherapy ranged from 2 to 8 days. After 6 weeks of chemotherapy, the average decrease in size of the largest tumor and the largest contralateral tumor was 41% (median 49%) and 47% (median 34%), respectively. In 2 cases where additional chemotherapy was given to further reduce tumor size preoperatively, the interval decrease in size of the largest tumor was 14-16% with no change in contralateral tumors; in one, all other ipsilateral tumors increased. Radical nephrectomy was required in 3 children on one side only; one whose multifocal tumors increased in size after additional chemotherapy, one who had the only mass to increase in size after initial chemotherapy (11.0 to 11.2 cm), and one who underwent conversion to radical nephrectomy due to an inability to spare the renal vein and collecting system. Renal function was preserved in all 3. In 2 kidneys, the largest tumors were reduced from 16.0 to 8.2 cm and from 6.7 to 3.0 cm; partial nephrectomies were achieved. Among all other partial nephrectomies, the largest tumor was 2.8 cm at diagnosis. Pathology confirmed Wilms tumor in all children. Only 2 who had pulmonary metastases at diagnosis had individual kidney staging greater than Stage I. After an average 5 months postoperative followup, monitoring of pulmonary lesions is ongoing in 3 children and 2 have no evidence of disease. CONCLUSIONS: In our series, biopsy-free management of bilateral Wilms tumors did not lead to misdiagnosis. While bilateral partial nephrectomies were achieved, neoadjuvant chemotherapy did not preclude radical nephrectomy in 3 children.