PMID- 31725643
OWN - NLM
STAT- MEDLINE
DCOM- 20191206
LR  - 20221005
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 98
IP  - 46
DP  - 2019 Nov
TI  - Childhood adrenocortical tumor: A clinical and immunohistochemical study of 13 
      cases.
PG  - e17921
LID - 10.1097/MD.0000000000017921 [doi]
LID - e17921
AB  - The aim of the study was to investigate the molecular mechanisms in childhood 
      adrenocortical tumors (ACTs), which is still unclear.A total of 9 girls and 4 
      boys with ACTs were enrolled. Relevant clinical features were obtained from 
      records. Immunohistochemistry of vimentin, chromogranin A, S100, synaptophysin, 
      cytokeratin (CK), type 2 3beta-hydroxysteroid dehydrogenase (3betaHSD), cytochrome 
      P45017alpha, p53, p21, p27, cyclin D1, Ki-67, insulin growth facter-2 (IGF-2), and 
      beta-catenin were undertaken for 13 tumors and 3 adjacent normal tissues. TP53 
      mutations in exon 2-11 were analyzed for 6 tumors and 3 blood 
      samples.Virilization was the most common presentation (8/13, 61.5%). 
      Immunohistochemically, p53 was positive in 8 of 13 ACTs and none in controls 
      while p21 was positive in 12 of 13 ACTs and none in controls (P = .0036). Ki-67 
      was positive in 10 of 13 ACTs, but not in normal tissues (P = .0089). Although 
      the expression of p27, cyclin D1, IGF-2 and beta-catenin were similar between the 
      ACTs and controls, beta-catenin was noted in nuclear of 3 ACTs but not in controls. 
      The difference of type 2 3betaHSD and P450c17alpha was not significant (P > .05, 
      respectively). Four variants of TP53 were identified in the 6 tumors. C215G 
      variant was found in 5 of 6 while A701G and G743A variants were found in 1 case, 
      respectively. A novel C680G variant was also noted in 1 case. It was notable that 
      C215G variant was found in the blood mononuclear cell of 3 patients.In 
      conclusion, p53 variant and p21 overexpression, and abnormal beta-catenin 
      distribution may be involved in the etiology and mechanism of childhood ACTs.
FAU - Wu, Xiaohui
AU  - Wu X
AD  - Children's Hospital of Zhejiang University School of Medicine.
FAU - Xu, Jun
AU  - Xu J
AD  - Children's Hospital of Zhejiang University School of Medicine.
AD  - Hangzhou Children's Hospital, Hangzhou, China.
FAU - Wang, Jinhu
AU  - Wang J
AD  - Children's Hospital of Zhejiang University School of Medicine.
FAU - Gu, Weizhong
AU  - Gu W
AD  - Children's Hospital of Zhejiang University School of Medicine.
FAU - Zou, Chaochun
AU  - Zou C
AD  - Children's Hospital of Zhejiang University School of Medicine.
LA  - eng
PT  - Journal Article
PT  - Observational Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Chromogranin A)
RN  - 0 (Ki-67 Antigen)
RN  - 0 (Poly-ADP-Ribose Binding Proteins)
RN  - 0 (Synaptophysin)
RN  - 0 (Vimentin)
RN  - 67763-97-7 (Insulin-Like Growth Factor II)
RN  - 68238-35-7 (Keratins)
RN  - EC 1.1.- (3-Hydroxysteroid Dehydrogenases)
SB  - IM
MH  - 3-Hydroxysteroid Dehydrogenases/biosynthesis
MH  - Adrenal Cortex Neoplasms/*epidemiology/*pathology/surgery
MH  - Age Factors
MH  - Child
MH  - Child, Preschool
MH  - Chromogranin A/biosynthesis
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - Infant
MH  - Insulin-Like Growth Factor II/biosynthesis
MH  - Keratins/biosynthesis
MH  - Ki-67 Antigen/biosynthesis
MH  - Male
MH  - Poly-ADP-Ribose Binding Proteins/biosynthesis
MH  - Sex Factors
MH  - Synaptophysin/biosynthesis
MH  - Vimentin/biosynthesis
MH  - Virilism/*epidemiology
PMC - PMC6867732
COIS- The authors have no conflicts of interests to disclose.
EDAT- 2019/11/15 06:00
MHDA- 2019/12/18 06:00
PMCR- 2019/11/15
CRDT- 2019/11/15 06:00
PHST- 2019/11/15 06:00 [entrez]
PHST- 2019/11/15 06:00 [pubmed]
PHST- 2019/12/18 06:00 [medline]
PHST- 2019/11/15 00:00 [pmc-release]
AID - 00005792-201911150-00045 [pii]
AID - MD-D-19-05329 [pii]
AID - 10.1097/MD.0000000000017921 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2019 Nov;98(46):e17921. doi: 10.1097/MD.0000000000017921.