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Clinical and Outcome Characteristics of Children With Adrenocortical Tumors: A Report From the International Pediatric Adrenocortical Tumor Registry

From the Oncologic Pediatric Surgery Division, Erasto Gaertner Hospital, Liga Paranaense de Combate ao CÂncer, Curitiba; Division of Pediatric Endocrinology and Center for Molecular Genetics and Childhood Cancer Research (CEGEMPAC), Hospital de Clinicas, Federal University of Paraná, Curitiba, Paraná; the Biostatistics Division, Hemocentro, and The Instituto Domingos Boldrini, Universidade Estadual de Campinas, Campinas; Department of Pediatric Oncology, Universidade Federal de São Paulo, Escola Paulista de Medicina; Department of Pediatrics, São Paulo University Medical School, Sao Paulo, Brazil; the Departments of Pathology, the Department of Hematology-Oncology, and the International Outreach Program, St Jude Children's Research Hospital; and the Department of Pediatrics, University of Tennessee College of Medicine, Memphis, TN.

Address reprint requests to Raul C. Ribeiro, MD, Department of Hematology-Oncology, St Jude Children's Research Hospital, 332 N Lauderdale St, Memphis, TN 38105-2794; e-mail: raul.ribeiro{at}stjude.org

PURPOSE: We created a registry for pediatric adrenocortical tumors (ACTs), which are rare and are not well characterized. We provide a descriptive analysis of 254 patients registered on the International Pediatric Adrenocortical Tumor Registry.

PATIENTS AND METHODS: Between January 1990 and December 2001, 254 patients younger than 20 years of age with newly diagnosed or previously treated ACTs were registered. A histologic diagnosis of ACT was required, although central review was not mandatory. Follow-up information was periodically requested from the referring physician. Treatment was chosen by the primary physician.

RESULTS: The overall female-male ratio was 1.6:1, but it varied widely among age groups. The most common presenting sign (84.2%) was virilization. Cushing's syndrome without virilization was uncommon (5.5%). Tumors were completely resected in 83% of patients. Patients with disseminated or residual disease received mitotane, cisplatin, etoposide, and/or doxorubicin, and rarely, radiation therapy. At a median follow-up of 2 years and 5 months, 157 patients (61.8%) survived without evidence of disease and 97 patients (38.2%) had died. The 5-year event-free survival estimate was 54.2% (95% CI, 48.2% to 60.2%). In a multivariate analysis, disease stage, presenting signs of endocrine dysfunction, and age were independently associated with prognosis.

CONCLUSION: Childhood ACTs occur predominantly in females and almost always causes clinical signs. Complete resection is required for cure. Residual or metastatic disease carries a poor prognosis. Our results demonstrate the feasibility of a disease-specific database for obtaining meaningful clinical and outcome information.

Supported in part by grants CA-21765 and CA-71907 from the National Institutes of Health (US Department of Health and Human Services), a Center of Excellence grant from the State of Tennessee, the Conselho Nacional de Pesquisa (CNPq) of Brazil, and the American Lebanese Syrian Associated Charities (ALSAC).

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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