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Renal Cell Carcinoma in Children: A Clinicopathologic Study

From the Pediatric Oncology Service-Pediatric Department II, University of Napoli, Napoli; Divisione di Urologia, Dipartimento di Terapia Chirurgica, Istituto Nazionale Tumori of Milano, Milano; Pediatric Department, University of Padova, Padova; Pediatric Oncology, University of Roma, and Division of Pediatric Oncology, Bambin Gesù Hospital, Roma; Pediatric Oncology, University of Bologna, Bologna; Pediatric Oncology University of Ancona, Ancona; Division of Pediatric Oncology Burlo Garofaeo Hospital of Trieste, Trieste; Giannina Gaslini Children’s Hospital of Genova, Genova; and Department of "Biomedicina dell’età evolutiva" University of Bari, Bari, Italy.Submitted February 14, 2002; accepted October 7, 2002.

Address reprint requests to Paolo Indolfi, MD, Pediatric Oncology Service-Pediatric Department II University of Napoli, Via S Andrea delle Dame, 4 80138 Napoli Italy; email: paolo.indolfi{at}unina2.it.

Purpose: To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC).

Patients and Methods: The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts.

Results: Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients.

Conclusion: RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.

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