Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions

Adrenocortical tumors (ACT) in children under 15 years of age exhibit some clinical and biological features distinct from ACT in adults. Cell proliferation, hypertrophy and cell death in adrenal cortex during the last months of gestation and the immediate postnatal period seem to be critical for the...

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Main Authors: Figueiredo B.C., Ribeiro R.C., Zambetti G., Haddad B., Pianovsky M.D., Pereira R.M., DeLacerda L., Sandrini R.
Format: Article
Language:English
Published: Associação Brasileira de Divulgação Científica 2000-01-01
Series:Brazilian Journal of Medical and Biological Research
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2000001000012
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author Figueiredo B.C.
Ribeiro R.C.
Zambetti G.
Haddad B.
Pianovsky M.D.
Pereira R.M.
DeLacerda L.
Sandrini R.
author_facet Figueiredo B.C.
Ribeiro R.C.
Zambetti G.
Haddad B.
Pianovsky M.D.
Pereira R.M.
DeLacerda L.
Sandrini R.
author_sort Figueiredo B.C.
collection DOAJ
description Adrenocortical tumors (ACT) in children under 15 years of age exhibit some clinical and biological features distinct from ACT in adults. Cell proliferation, hypertrophy and cell death in adrenal cortex during the last months of gestation and the immediate postnatal period seem to be critical for the origin of ACT in children. Studies with large numbers of patients with childhood ACT have indicated a median age at diagnosis of about 4 years. In our institution, the median age was 3 years and 5 months, while the median age for first signs and symptoms was 2 years and 5 months (N = 72). Using the comparative genomic hybridization technique, we have reported a high frequency of 9q34 amplification in adenomas and carcinomas. This finding has been confirmed more recently by investigators in England. The lower socioeconomic status, the distinctive ethnic groups and all the regional differences in Southern Brazil in relation to patients in England indicate that these differences are not important to determine 9q34 amplification. Candidate amplified genes mapped to this locus are currently being investigated and Southern blot results obtained so far have discarded amplification of the abl oncogene. Amplification of 9q34 has not been found to be related to tumor size, staging, or malignant histopathological features, nor does it seem to be responsible for the higher incidence of ACT observed in Southern Brazil, but could be related to an ACT from embryonic origin.
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spelling doaj.art-045d6d6ac45a4ae0b095f04acd9875d32022-12-21T19:24:37ZengAssociação Brasileira de Divulgação CientíficaBrazilian Journal of Medical and Biological Research0100-879X0034-73102000-01-01331012171224Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditionsFigueiredo B.C.Ribeiro R.C.Zambetti G.Haddad B.Pianovsky M.D.Pereira R.M.DeLacerda L.Sandrini R.Adrenocortical tumors (ACT) in children under 15 years of age exhibit some clinical and biological features distinct from ACT in adults. Cell proliferation, hypertrophy and cell death in adrenal cortex during the last months of gestation and the immediate postnatal period seem to be critical for the origin of ACT in children. Studies with large numbers of patients with childhood ACT have indicated a median age at diagnosis of about 4 years. In our institution, the median age was 3 years and 5 months, while the median age for first signs and symptoms was 2 years and 5 months (N = 72). Using the comparative genomic hybridization technique, we have reported a high frequency of 9q34 amplification in adenomas and carcinomas. This finding has been confirmed more recently by investigators in England. The lower socioeconomic status, the distinctive ethnic groups and all the regional differences in Southern Brazil in relation to patients in England indicate that these differences are not important to determine 9q34 amplification. Candidate amplified genes mapped to this locus are currently being investigated and Southern blot results obtained so far have discarded amplification of the abl oncogene. Amplification of 9q34 has not been found to be related to tumor size, staging, or malignant histopathological features, nor does it seem to be responsible for the higher incidence of ACT observed in Southern Brazil, but could be related to an ACT from embryonic origin.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2000001000012adrenocortical carcinomacancermutation9q34 amplification
spellingShingle Figueiredo B.C.
Ribeiro R.C.
Zambetti G.
Haddad B.
Pianovsky M.D.
Pereira R.M.
DeLacerda L.
Sandrini R.
Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions
Brazilian Journal of Medical and Biological Research
adrenocortical carcinoma
cancer
mutation
9q34 amplification
title Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions
title_full Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions
title_fullStr Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions
title_full_unstemmed Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions
title_short Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions
title_sort amplification of 9q34 in childhood adrenocortical tumors a specific feature unrelated to ethnic origin or living conditions
topic adrenocortical carcinoma
cancer
mutation
9q34 amplification
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2000001000012
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