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Cited by 146Adrenal and Paraganglionic TumorsSarcoma Diagnosis and TreatmentNeuroblastoma Research and Treatments

Biology, clinical characteristics, and management of adrenocortical tumors in children

Rodriguez‐Galindo, Carlos, Figueiredo, Bonald C., Zambetti, Gerard P., Ribeiro, Raul C.·2005·Pediatric Blood & Cancer
DOI 10.1002/pbc.20318
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Abstract

Childhood adrenocortical tumors (ACT) are very aggressive endocrine neoplasms whose incidence is quite low. Little is known about their pathogenesis, clinical presentation, and optimal treatment. In recent years, however, new information has been derived from the International Pediatric Adrenocortical Tumor Registry (IPACTR), and new clues to its pathogenesis have emerged. To provide an overview of the available data that may apply to pediatric ACT, we reviewed the epidemiology, pathogenesis, and treatment of ACT in adults and in children. Germline TP53 mutation is almost always the predisposing factor in childhood ACT. A unique germline mutation (TP53-R337H) has been described in Southern Brazil, where the incidence of ACT is 10-15 times the general incidence. Childhood ACT typically present during the first 5 years of life and has female predominance. Hormone hyperproduction is almost universal, and most patients present with virilization. Two-thirds of patients have resectable tumors. Surgery is the definitive treatment for ACT, and a curative complete resection should always be attempted. Cisplatin-based chemotherapy with mitotane is indicated for unresectable or metastatic disease, although its impact on overall outcome is slight. In childhood ACT, age, tumor size, and tumor resectability are the most important prognostic indicators. Outcome is stage-dependent; patients with small, resectable tumors have survival rates in excess of 80%, whereas the outcome for patients with unresectable disease is dismal. Patients with large, resectable tumors have an intermediate outcome. Childhood ACT are rare, but their unique epidemiology appear to implicate novel oncogenic pathways that are unique to the pediatric population. Multi-institutional and prospective studies are necessary to further our understanding of the pathogenesis and to improve outcomes.

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Crossref · 10.1002/pbc.20318

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  • The relative increase in childhood ACC incidence is mainly explained by germline TP53 mutations, which are the underlying genetic cause of ACC in ∼50% to 80% of children with ACC.

    “The relative increase in incidence in childhood is mainly explained by germline TP53 mutations, which are the underlying genetic cause of ACC in ∼50% to 80% of children with ACC (Table 1)(28–30).”

    From Adrenocortical carcinoma (2014) · cites this paper as [28]

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Authors
Rodriguez‐Galindo, C., Figueiredo, B. C., Zambetti, G. P., & Ribeiro, R. C.
Affiliations
  • Hospital de Clínicas Universidade Federal do Paraná (BR)
  • St. Jude Children's Research Hospital (US)
ORCID
Venue
Pediatric Blood & Cancer
Publisher
Wiley
Published
2005-03-03
Volume
45
Issue
3
Pages
265-273
ISSN
  • 1545-5009 (ISSN-L)
  • 1545-5017
DOI
10.1002/pbc.20318
OpenAlex
W2013170178
Cited by
146
References
85
Citations by year
2026: 1 · 2025: 7 · 2024: 9 · 2023: 9 · 2022: 9 · 2021: 16 · 2020: 10 · 2019: 3 · 2018: 9 · 2017: 5 · 2016: 9 · 2015: 5 · 2014: 11 · 2013: 5 · 2012: 8
FWCI
0.81
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Pediatric Blood & Cancer (publishedVersion)
  • PubMed (publishedVersion)
Funders
National Cancer Institute (CA-71907, CA21765, P30CA021765, P01CA071907)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Sarcoma Diagnosis and Treatment
  • Health Sciences › Medicine › Neurology › Neuroblastoma Research and Treatments
Keywords
pediatric adrenocortical tumors, TP53 mutation, germline predisposition, masculinization, hormone hypersecretion, adrenalectomy, mitotane, cisplatin-based chemotherapy, prognostic factors, carcinogenesis, metastatic disease
MeSH terms
Adolescent, Adrenal Cortex Neoplasms, Child, Child, Preschool, Humans, Infant, Mitotane, Mutation, Neoplasm Staging, Research, Survival Rate, Genes, p53, Antineoplastic Agents, Hormonal

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