PMID- 23320395
OWN - NLM
STAT- MEDLINE
DCOM- 20130627
LR  - 20221207
IS  - 1746-1596 (Electronic)
IS  - 1746-1596 (Linking)
VI  - 8
DP  - 2013 Jan 15
TI  - Copy number gain of MYCN gene is a recurrent genetic aberration and favorable 
      prognostic factor in Chinese pediatric neuroblastoma patients.
PG  - 5
LID - 10.1186/1746-1596-8-5 [doi]
AB  - BACKGROUND: Amplification of MYCN oncogene is an established marker indicating 
      aggressive tumor progression of neuroblastoma (NBL). But copy number analyses of 
      MYCN gene in ganglioneuroblastoma (GNBL) and ganglioneuroma(GN) is poorly 
      described in the literature. In the study, we evaluated the copy number 
      aberrations of MYCN gene in clinical samples of NBLs, GNBLs and GNs and analyzed 
      their association with clinical outcome of the patients. METHODS: In this study, 
      we analyzed MYCN gene and chromosome 2 aneusomy by using fluorescence in situ 
      hybridization (FISH) method in a total of 220 patients with NBL, GNBL and GN 
      cases. Kaplan-Meier curves were generated by using SPSS 12.0 software. RESULTS: 
      Of 220 patients, 178 (81.0%) were NBLs, 32 (14.5%) were GNBLs and 10 (4.5%) were 
      GNs. MYCN gain is a recurrent genetic aberration of neuroblastic tumors (71.8%, 
      158/220), which was found in 129 NBLs (58.6%, 129/220), 25 GNBLs (11.4%, 25/220) 
      and 4 GN cases (1.8%, 4/220). However, MYCN amplification was only present in 24 
      NBL tumors (13.5%, 24/178) and 1 GNBL case (3.1%, 1/32). Kaplan-Meier survival 
      analysis indicated that MYCN amplification is significantly correlated with 
      decreased overall survival in NBLs (P=0.017). Furthermore, a better prognosis 
      trend was observed in patients with MYCN gain tumors compared with those with 
      MYCN gene normal copy number tumors and MYCN amplification tumors (P=0.012). 
      CONCLUSIONS: In summary, the frequency of MYCN amplification in NBLs is high and 
      is rarely observed in GNBLs and GNs, which suggest MYCN plays an important role 
      in neuroblastic tumors differentiation. MYCN gain appeared to define a subgroup 
      of NBLs with much better outcome and classification of MYCN gene copy number 
      alteration as three groups (amplification, gain and normal) can provide a 
      powerful prognostic indicator in NBLs. VIRTUAL SLIDES: The virtual slide(s) for 
      this article can be found here: 
      http://www.diagnosticpathology.diagnomx.eu/vs/6417541528559124.
FAU - Wang, Miao
AU  - Wang M
AD  - Department of Pathology, Basic Medical College, Capital Medical University, 
      Beijing, China.
FAU - Zhou, Chunju
AU  - Zhou C
FAU - Cai, Rongqin
AU  - Cai R
FAU - Li, Yong
AU  - Li Y
FAU - Gong, Liping
AU  - Gong L
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20130115
PL  - England
TA  - Diagn Pathol
JT  - Diagnostic pathology
JID - 101251558
RN  - 0 (MYCN protein, human)
RN  - 0 (N-Myc Proto-Oncogene Protein)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Oncogene Proteins)
SB  - IM
MH  - Asian People/genetics
MH  - Chi-Square Distribution
MH  - Child, Preschool
MH  - China/epidemiology
MH  - Chromosomes, Human, Pair 2
MH  - *DNA Copy Number Variations
MH  - Female
MH  - Ganglioneuroblastoma/ethnology/genetics/mortality
MH  - Ganglioneuroma/ethnology/genetics/mortality
MH  - *Gene Amplification
MH  - *Gene Dosage
MH  - Genetic Predisposition to Disease
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Kaplan-Meier Estimate
MH  - Male
MH  - N-Myc Proto-Oncogene Protein
MH  - Neuroblastoma/ethnology/*genetics/mortality
MH  - Nuclear Proteins/*genetics
MH  - Oncogene Proteins/*genetics
MH  - Phenotype
MH  - Prognosis
MH  - Time Factors
PMC - PMC3567989
EDAT- 2013/01/17 06:00
MHDA- 2013/06/29 06:00
PMCR- 2013/01/15
CRDT- 2013/01/17 06:00
PHST- 2012/11/24 00:00 [received]
PHST- 2013/01/08 00:00 [accepted]
PHST- 2013/01/17 06:00 [entrez]
PHST- 2013/01/17 06:00 [pubmed]
PHST- 2013/06/29 06:00 [medline]
PHST- 2013/01/15 00:00 [pmc-release]
AID - 1746-1596-8-5 [pii]
AID - 10.1186/1746-1596-8-5 [doi]
PST - epublish
SO  - Diagn Pathol. 2013 Jan 15;8:5. doi: 10.1186/1746-1596-8-5.