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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Pediatric adrenocortical tumor - review and management update

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Author(s):
Brondani, Vania B. [1] ; Fragoso, V, Maria Candida B.
Total Authors: 2
Affiliation:
[1] V, Univ Sao Paulo, Fac Med, Serv Endocrinol & Metabol, Hosp Clin, Unidade Suprarrenal, Lab Hormonios & Gen, Av Dr Eneas de Carvalho Aguiar 155, Sao Paulo - Brazil
Total Affiliations: 1
Document type: Review article
Source: CURRENT OPINION IN ENDOCRINOLOGY DIABETES AND OBESITY; v. 27, n. 3, p. 177-186, JUN 2020.
Web of Science Citations: 0
Abstract

Purpose of review Adrenocortical tumor (ACT) is a rare disease with an annual worldwide incidence of 0.3-0.38/million children below 15 years old, and Brazilian population presents the highest incidence because of germline mutation in theTP53. Pediatric ACT is associated with virilizing features and hypercortisolism in most cases. Malignancy is defined when local invasion or metastasis is found, and it is associated with a poor prognosis. However, the correct and early diagnosis and treatment may impact on overall and disease-free survival. Recent findings A complete understanding of the disease and its singularities facilitates the assistance to the pediatric patient with ACT. The new insights about adrenal tumorigenesis have provided a better understanding of this disease. In this scenario, the era of molecular studies is leading to the refinement of the taxonomy, and it is offering the opportunity to discover new biomarkers and pathways of tumorigenesis, beyond the knowing beta-catenin, Insulin-like growth factor-II/IGF-IR, and the p53/Rb signaling. The rarity of this disease makes it a real challenge. Here, we present a review focusing on clinical practice. A methodic approach aiming to clarify the diagnosis and a follow-up are suggested to guide physicians in the assistance of pediatrics patients, improving the prognosis. (AU)

FAPESP's process: 17/26345-5 - Evaluation of prognostic factors of adrenal cortex tumors and predictors of response to treatment with mitotane in carcinomas
Grantee:Maria Candida Barisson Villares Fragoso
Support Opportunities: Regular Research Grants