Wang, L LTeshiba, RIkegaki, NTang, X XNaranjo, ALondon, W BHogarty, M DGastier-Foster, J MLook, A TPark, J RMaris, J MCohn, S LSeeger, R CAsgharzadeh, SShimada, H2016-07-142015Wang, L. L., R. Teshiba, N. Ikegaki, X. X. Tang, A. Naranjo, W. B. London, M. D. Hogarty, et al. 2015. “Augmented expression of MYC and/or MYCN protein defines highly aggressive MYC-driven neuroblastoma: a Children's Oncology Group study.” British Journal of Cancer 113 (1): 57-63. doi:10.1038/bjc.2015.188. http://dx.doi.org/10.1038/bjc.2015.188.0007-0920http://nrs.harvard.edu/urn-3:HUL.InstRepos:27662175Background: MYCN amplification with subsequent MYCN protein overexpression is a powerful indicator of poor prognosis of neuroblastoma patients. Little is known regarding the prognostic significance of the homologous MYC protein expression in neuroblastoma. Methods: Immunostaining for MYCN and MYC protein was performed on 357 undifferentiated/poorly differentiated neuroblastomas. Results were analysed with other prognostic markers. Results: Sixty-seven (19%) tumours were MYCN(+), 38 (11%) were MYC(+), and one(0.3%) had both proteins(+). MYCN(+) tumours and MYC(+) tumours were more likely diagnosed in children>18months with stage4-disease. MYCN(+) tumours were associated with amplified MYCN, Unfavourable Histology (UH), and High-MKI (Mitosis–Karyorrhexis Index). MYC(+) tumours were also frequently UH but not associated with MYCN amplification, and more likely to have low-/intermediate-MKI. Favourable Histology patients without MYC/MYCN expressions exhibited the best survival (N=167, 89.7±5.5% 3-year EFS, 97.0±3.2% 3-year OS), followed by UH patients without MYC/MYCN expressions (N=84, 63.1±13.6% 3-year EFS, 83.5±9.4% 3-year OS). MYCN(+)patients and MYC(+)patients had similar and significantly low (P<0.0001) survivals (46.2±12.0% 3-year EFS, 63.2±12.1% 3-year OS and 43.4±23.1% 3-year EFS, 63.5±19.2% 3-year OS, respectively). Notably, the prognostic impact imparted by MYC expression was independent from other markers. Conclusions: In this series, ∼30% of neuroblastomas had augmented MYCN or MYC expression with dismal survivals. Prospective study of MYC/MYCN protein expression signature as a new biomarker for high-risk neuroblastomas should be conducted.en-USneuroblastomaMYCN proteinMYC proteinimmunohistochemistryprognosisJournal Article2016-07-1410.1038/bjc.2015.188