The DOI matches its Crossref record; 37 of the 37 references it cites were found in a registry or an index.
Clinical and Outcome Characteristics of Children With Adrenocortical Tumors: A Report From the International Pediatric Adrenocortical Tumor Registry
Open accessAbstract
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.
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“In the adult as well as in the pediatric population, there is a predilection for the female gender (the ratio of female to male ranges from 1.5–2.5:1) (10, 17).”
From Adrenocortical carcinoma (2014) · cites this paper as [17]
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“The fact that 1.3% of all childhood cancers are ACCs as opposed to 0.02% to 0.2% of adult cancers confirms a higher relative incidence early in life (12, 15–17).”
From Adrenocortical carcinoma (2014) · cites this paper as [17]
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Metadata
- Authors
- Michalkiewicz, E., Sandrini, R., Figueiredo, B., Miranda, E. C. M., Caran, E., Oliveira-Filho, A. G., Marques, R., Pianovski, M. A. D., Lacerda, L., Cristofani, L. M., Jenkins, J., Rodriguez-Galindo, C., & Ribeiro, R. C.
- Affiliations
- Universidade Estadual de Campinas (UNICAMP) (BR)
- St. Jude Children's Research Hospital (US)
- 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...
- Corresponding author
- Ribeiro, R.C.
- Venue
- Journal of Clinical Oncology
- Published
- 2004-03-01
- Volume
- 22
- Issue
- 5
- Pages
- 838-845
- ISSN
- 0732-183X (ISSN-L)
- 1527-7755
- OpenAlex
- W2120218419
- Cited by
- 519
- References
- 37
- Citations by year
- 2026: 11 · 2025: 16 · 2024: 29 · 2023: 28 · 2022: 34 · 2021: 35 · 2020: 29 · 2019: 26 · 2018: 24 · 2017: 20 · 2016: 33 · 2015: 27 · 2014: 35 · 2013: 21 · 2012: 22
- FWCI
- 12.67
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Clinical Oncology (publishedVersion)
- PubMed (publishedVersion)
- UNIFESP Institutional Repository (Universidade Federal de São Paulo) (submittedVersion)
- Funders
- U.S. Department of Health and Human Services
- American Lebanese Syrian Associated Charities (CA 21765)
- Conselho Nacional de Desenvolvimento Científico e Tecnológico
- National Institutes of Health (CA21765))
- National Cancer Institute (CA-71907, CA21765, P30CA021765, P01CA071907)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Oncology › Neuroblastoma Research and Treatments
- Health Sciences › Medicine › Oncology › Sarcoma Diagnosis and Treatment
- Keywords
- pediatric adrenocortical tumors, adrenocortical carcinoma, masculinization, Cushing's syndrome, surgical resection, mitotane, chemotherapy, event-free survival, prognosis, disease stage, sex differences, rare pediatric tumors
- MeSH terms
- Adolescent, Adrenal Cortex Neoplasms, Adrenalectomy, Adult, Age Factors, Biopsy, Needle, Child, Child, Preschool, Combined Modality Therapy, Female, Humans, International Cooperation, Male, Neoplasm Staging, Probability, Prognosis, Registries, Retrospective Studies, Sex Factors, Confidence Intervals, Proportional Hazards Models, Survival Analysis, Treatment Outcome, Chemotherapy, Adjuvant, Adrenocortical Carcinoma, Risk Assessment, Disease-Free Survival
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