@entity1636 ( @entity1636 ) is a rare malignancy with poor prognosis. The incidence of pediatric @entity16663 ( @entity16663 ) is remarkably high in Southern Brazil, where it is estimated to be 15 times greater than the world occurrence, due to a high frequency of a germline mutation ( @entity28503 ) of the @entity876 gene. Differently from adults, pediatric @entity1636 with apparently poor prognosis based on histopathological features have often a good clinical outcome. A high Weiss score is definitely not a good predictor of survival in @entity1 , but it is much more discriminative of a poor outcome in @entity5 . Besides important differences in prognosis, adrenocortical tumorigenesis has distinct patterns between @entity1 and adults. In this review, we summarize recent data from ours and other Institutions, showing that the prognostic importance of molecular markers is striking different between pediatric and adult @entity16663 . Although the majority of pediatric @entity16663 are associated with @entity28503 germline mutation, it is not a predictor of poor outcome in @entity1 and adolescents with @entity16663 . On the other side, @entity876 somatic mutations define a subgroup of adult @entity1636 with different tumorigenesis and unfavorable prognosis. IGF system has a central role in the malignant phenotype of @entity16663 , but in @entity5 it is mediated by @entity5100 over-expression and in @entity5 by @entity2856 over-expression. Finally, @entity25626 over-expression is associated with decreased overall survival and recurrence-free survival in adult @entity1636 , but not in the pediatric group. In conclusion, discriminating benign and malignant behavior is more challenging in pediatric @entity16663 than in @entity5 .

Differences in the molecular mechanisms of adrenocortical tumorigenesis between XXXX and adults.

@entity1 :: ('9606', 'Species') :: ['children'] @entity5 :: ('MESH:D009369', 'Disease') :: ['adult tumors', 'pediatric tumors'] @entity5100 :: ('3481', 'Gene') :: ['IGF2'] @entity876 :: ('7157', 'Gene') :: ['TP53'] @entity16663 :: ('MESH:D000306', 'Disease') :: ['adrenocortical tumors', 'ACT'] @entity1636 :: ('MESH:D018268', 'Disease') :: ['Adrenocortical carcinoma', 'ACC', 'adrenocortical neoplasms'] @entity2856 :: ('3480', 'Gene') :: ['IGF1R'] @entity28503 :: ('p|SUB|R|337|H', 'ProteinMutation') :: ['p.R337H'] @entity25626 :: ('7536', 'Gene') :: ['SF1']
@entity1 :: (9606,Species) :: ['children']
