Correlation between the International Neuroblastoma Pathology Classification and genomic signature in neuroblastoma.
Bottom Line: Overall survival was not significantly different among the genomic groups in FH; however, it was inferior in UH with GGP.In UH with a single copy MYCN, genomic subgroups GGP2s (both 1p and 11q losses) and GGP3s (partial 11q loss but not 1p loss) indicated significantly poor prognosis compared to GGP4s (no partial 1p and 11q loss).As INPC and MYCN amplification were found to be the most powerful prognostic biological factors, they should be included with genomic grouping as treatment stratification for patients with UH and single copy of MYCN.
Affiliation: Department of Pathology, National Center for Child Health and Development, Tokyo, Japan.Show MeSH
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Mentions: As MYCN amplification is a known indicator of poor prognosis, we focused on NB without MYCN amplification, that is, NB with single copy MYCN. Both UH and GGP were significantly associated with poor prognosis in NB with single copy MYCN, as well as in all other types of NB (Fig.2). The survival rate was not significantly different among genomic groups in FH NB, but in UH NB, it was inferior in GGP compared to GGW/GGS (Fig.3). Next, we analyzed the survival rate among GGP subtypes in UH NB. The GGP2s and GGP3s subtypes showed significantly poorer prognosis (OS, 16.7%) than GGP4s (OS, 80%) which, together with GGWs, had better survival rate (P = 0.02) (Fig.4) (Table1). MYCN amplification was found to have no prognostic impact on UH NB with GGP; OS of MYCN amplified NB was 40.0%, whereas OS of MYCN non-amplified NB was 35.3% (P = 0.84) (Fig.5). In GGP with single copy MYCN (GGPs) subtypes, there was no significant prognostic difference between the presence and absence of pleomorphic cells (OS, 38% vs 50%, P = 0.481). Among the 15 GGPs cases having pleomorphic cells, 10 cases were GGP3s, 3 were GGP4s, and 2 were GGP2s. Pleomorphic cells were observed most prominently in GGP2s and GGP3s cases compared to GGP4s cases (P = 0.002) (Fig.6).
Affiliation: Department of Pathology, National Center for Child Health and Development, Tokyo, Japan.