Clinical and outcome characteristics of children with adrenocortical tumors: A report from the international pediatric adrenocortical tumor registry

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dc.contributor.author Michalkiewicz, E.
dc.contributor.author Sandrini, R.
dc.contributor.author Figueiredo, B.
dc.contributor.author Miranda, ECM
dc.contributor.author Caran, E.
dc.contributor.author Oliveira-Filho, A. G.
dc.contributor.author Marques, R.
dc.contributor.author Pianovski, MAD
dc.contributor.author Lacerda, L.
dc.contributor.author Cristofani, L. M.
dc.contributor.author Jenkins, J.
dc.contributor.author Rodriguez-Galindo, C.
dc.contributor.author Ribeiro, R. C.
dc.date.accessioned 2016-01-24T12:37:02Z
dc.date.available 2016-01-24T12:37:02Z
dc.date.issued 2004-03-01
dc.identifier http://dx.doi.org/10.1200/JCO.2004.08.085
dc.identifier.citation Journal of Clinical Oncology. Alexandria: Amer Soc Clinical Oncology, v. 22, n. 5, p. 838-845, 2004.
dc.identifier.issn 0732-183X
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/27667
dc.description.abstract 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% Cl, 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.(C) 2004 by American Society of Clinical Oncology. en
dc.format.extent 838-845
dc.language.iso eng
dc.publisher Amer Soc Clinical Oncology
dc.relation.ispartof Journal of Clinical Oncology
dc.rights Acesso restrito
dc.title Clinical and outcome characteristics of children with adrenocortical tumors: A report from the international pediatric adrenocortical tumor registry en
dc.type Artigo
dc.contributor.institution St Jude Childrens Res Hosp
dc.contributor.institution Erasto Gaertner Hosp
dc.contributor.institution Univ Fed Parana
dc.contributor.institution Universidade Estadual de Campinas (UNICAMP)
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.contributor.institution Universidade de São Paulo (USP)
dc.contributor.institution Univ Tennessee
dc.description.affiliation St Jude Childrens Res Hosp, Dept Hematol Oncol, Memphis, TN 38105 USA
dc.description.affiliation St Jude Childrens Res Hosp, Int Outreach Program, Memphis, TN 38105 USA
dc.description.affiliation Erasto Gaertner Hosp, Oncol Pediat Surg Div, Curitiba, Parana, Brazil
dc.description.affiliation Univ Fed Parana, Hosp Clin, Div Pediat Endocrinol, BR-80060000 Curitiba, Parana, Brazil
dc.description.affiliation Univ Fed Parana, Hosp Clin, CEGEMPAC, Ctr Mol Genet & Childhood Canc Res, BR-80060000 Curitiba, Parana, Brazil
dc.description.affiliation Univ Estadual Campinas, Hemoctr, Div Biostat, BR-13081970 Campinas, SP, Brazil
dc.description.affiliation Univ Estadual Campinas, Inst Domingos Boldrini, BR-13081970 Campinas, SP, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Escola Paulista Med, Dept Pediat Oncol, São Paulo, Brazil
dc.description.affiliation Univ São Paulo, Sch Med, Dept Pediat, BR-05508 São Paulo, Brazil
dc.description.affiliation St Jude Childrens Res Hosp, Dept Pathol, Memphis, TN 38105 USA
dc.description.affiliation Univ Tennessee, Coll Med, Dept Pediat, Memphis, TN USA
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Escola Paulista Med, Dept Pediat Oncol, São Paulo, Brazil
dc.identifier.doi 10.1200/JCO.2004.08.085
dc.description.source Web of Science
dc.identifier.wos WOS:000189380900013



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