Copy number gain of <it>MYCN</it> gene is a recurrent genetic aberration and favorable prognostic factor in Chinese pediatric neuroblastoma patients

• Main Authors: Wang Miao (Author), Zhou Chunju (Author), Cai Rongqin (Author), Li Yong (Author), Gong Liping (Author)
• Format: Book
• Published: BMC, 2013-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Summary

<p>Abstract</p> <p>Background</p> <p>Amplification of <it>MYCN</it> oncogene is an established marker indicating aggressive tumor progression of neuroblastoma (NBL). But copy number analyses of <it>MYCN</it> gene in ganglioneuroblastoma (GNBL) and ganglioneuroma(GN) is poorly described in the literature. In the study, we evaluated the copy number aberrations of <it>MYCN</it> gene in clinical samples of NBLs, GNBLs and GNs and analyzed their association with clinical outcome of the patients.</p> <p>Methods</p> <p>In this study, we analyzed <it>MYCN</it> 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.</p> <p>Results</p> <p>Of 220 patients, 178 (81.0%) were NBLs, 32 (14.5%) were GNBLs and 10 (4.5%) were GNs. <it>MYCN</it> 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, <it>MYCN</it> 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 <it>MYCN</it> amplification is significantly correlated with decreased overall survival in NBLs (P=0.017). Furthermore, a better prognosis trend was observed in patients with <it>MYCN</it> gain tumors compared with those with <it>MYCN</it> gene normal copy number tumors and <it>MYCN</it> amplification tumors (P=0.012).</p> <p>Conclusions</p> <p>In summary, the frequency of <it>MYCN</it> amplification in NBLs is high and is rarely observed in GNBLs and GNs, which suggest <it>MYCN</it> plays an important role in neuroblastic tumors differentiation. <it>MYCN</it> gain appeared to define a subgroup of NBLs with much better outcome and classification of <it>MYCN</it> gene copy number alteration as three groups (amplification, gain and normal) can provide a powerful prognostic indicator in NBLs.</p> <p>Virtual Slides</p> <p>The virtual slide(s) for this article can be found here: <url>http://www.diagnosticpathology.diagnomx.eu/vs/6417541528559124</url></p>

Item Description

10.1186/1746-1596-8-5
1746-1596