Key result
Underexpression of the TNF gene in childhood adrenocortical tumors was associated with lower event-free survival (P<0.01), and lower BCL2 expression was associated with death (P=0.04).
Why the study?
Does the expression of apoptosis-related genes correlate with prognosis in childhood adrenocortical tumors?
Observational (n=71)
Does the expression of apoptosis-related genes correlate with prognosis in childhood adrenocortical tumors?
p-value: p=<0.01
Low expression of BCL2 and TNF genes is associated with poor prognosis and lower event-free survival in childhood adrenocortical tumors.
May support risk stratification in childhood adrenocortical tumors; leaves open prospective validation before clinical use.
BACKGROUND: Impaired apoptosis has been implicated in the development of childhood adrenocortical tumors (ACT), although the expression of apoptosis-related gene expression in such tumors has not been reported. METHODS: The mRNA expression levels of the genes CASP3, CASP8, CASP9, FAS, TNF, NFKB, and BCL2 were analyzed by quantitative real-time PCR in consecutive tumor samples obtained at diagnosis from 60 children with a diagnosis of ACT and in 11 non-neoplastic adrenal samples. BCL2 and TNF protein expression was analyzed by immunohistochemistry. RESULTS: A significant association was observed between tumor size ≥100 g and lower expression levels of the BCL2 (P=0.03) and TNF (P=0.05) genes; between stage IV and lower expression levels of CASP3 (P=0.008), CASP9 (P=0.02), BCL2 (P=0.002), TNF (P=0.05), and NFKB (P=0.03); Weiss score ≥3 and lower expression of TNF (P=0.01); unfavorable event and higher expression values of CASP9 (P=0.01) and lower values of TNF (P=0.02); and death and lower expression of BCL2 (P=0.04). Underexpression of TNF was associated with lower event-free survival in uni- and multivariate analyses (P<0.01). Similar results were observed when patients with Weiss score <3 were excluded. CONCLUSION: This study supports the participation of apoptosis-related genes in the biology and prognosis of childhood ACT and suggests the complex role of these genes in the pathogenesis of this tumor.
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Lorea et al. (2012) conducted an observational in Childhood adrenocortical tumors (n=71). Underexpression of apoptosis-related genes (BCL2 and TNF) vs. Higher expression levels was evaluated on Event-free survival (p=<0.01). Underexpression of the TNF gene in childhood adrenocortical tumors was associated with lower event-free survival (P<0.01), and lower BCL2 expression was associated with death (P=0.04).
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