Clinical Characteristics and Long-Term Outcomes of Adrenal Tumors in Children and Adolescents.
Exp Clin Endocrinol Diabetes
; 131(10): 515-522, 2023 Oct.
Article
em En
| MEDLINE
| ID: mdl-37437600
ABSTRACT
OBJECTIVE:
Adrenal tumors are generally rare in children and can be a part of familial cancer syndrome. This research was conducted to examine the clinical outcomes, histopathological results, and genetic etiologies of adrenal tumors in children and adolescents.METHODS:
Thirty-one children and adolescents with adrenal tumors were included. Data on clinical outcomes and endocrine and radiologic results were retrospectively analyzed. Molecular analysis was conducted in select patients according to their phenotype and family history.RESULTS:
The median age at diagnosis was 7.9 years (range 0.8-17.8 years) with 5.1±1.8 cm of maximum tumor diameter. Adrenal adenoma (n=7), carcinoma (n=5), borderline (n=2), isolated micronodular adrenocortical disease (n=2), pheochromocytoma (n=8), paraganglioma (n=3), and ganglioneuroma (n=4) are all pathological diagnoses. The most common presenting symptom was excess production of adrenocortical hormones (n=15), including virilization and Cushing syndrome. Non-functioning adrenocortical tumors were found in a patient with congenital adrenal hyperplasia. Genetic etiologies were identified in TP53 (n=5), VHL (n=4), and PRKACA (n=1). Patients with mutations in TP53 were young (1.5±0.5 years) and had large masses (6.1±2.3 cm).CONCLUSIONS:
This study describes clinical outcomes and the pathological spectrum of adrenal tumors in children and adolescents. Adrenocortical tumors mostly presented with an excess of the adrenocortical hormone. Patients with genetic defects presented at a young age and large size of tumors, necessitating genetic testing in patients at a young age.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias do Córtex Suprarrenal
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Neoplasias das Glândulas Suprarrenais
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Adenoma Adrenocortical
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Síndrome de Cushing
Tipo de estudo:
Etiology_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Child
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Child, preschool
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Humans
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Infant
Idioma:
En
Revista:
Exp Clin Endocrinol Diabetes
Assunto da revista:
ENDOCRINOLOGIA
Ano de publicação:
2023
Tipo de documento:
Article