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Neuroblastoma of undifferentiated subtype, prognostic significance of prominent nucleolar formation, and MYC/MYCN protein expression: a report from the Children's Oncology Group.

Abstract：

BACKGROUND:This study sought to investigate biological/clinicopathological characteristics of neuroblastoma, undifferentiated subtype (NBUD). METHODS:This study examined 157 NBUD cases filed at the Children's Oncology Group Neuroblastoma Pathology Reference Laboratory, and survival rates of the patients were analyzed with known prognostic factors. Immunostainings for MYCN and MYC protein were performed on 68 tumors. RESULTS:NBUD cases had a poor prognosis (48.4% ± 5.0% 3-year event-free survival [EFS]; 56.5% ± 5.0% overall survival [OS]), and were often associated with high mitosis-karyorrhexis index (MKI, 65%), prominent nucleoli (PN, 83%), ≥ 18 months of age (75%), MYCN amplification (MYCN-A, 83%), diploid pattern (63%), and 1pLOH (loss of heterozygosity (72%). However, these prognostic indicators, except for MYCN status, had no significant impact on survival. Surprisingly, EFS for patients with MYCN-A tumors (53.4% ± 5.6%) was significantly better (P=.0248) than for patients with MYCN-nonamplified (MYCN-NA) tumors (31.7% ± 11.7%), with MYCN-NA and PN (+) tumors having the worst prognosis (9.3% ± 8.8%, P=.0045). Immunohistochemically, MYCN expression was found in 42 of 48 MYCN-A tumors. In contrast, MYC expression was almost exclusively found in the MYCN-NA tumors (9 of 20) especially when they had PN (8 of 11). Those patients with only MYC-positive tumors had the worst EFS (N=8, 12.5% ± 11.7%) compared with only MYCN-positive (N=39, 49.9% ± 17.7%) and both negative tumors (N=15, 70.0% ± 17.1%) (P= .0029). High MKI was often found in only MYCN-positive (30 of 38) but rarely in only MYC-positive (2 of 8) tumors. CONCLUSIONS:NBUD represents a unique subtype of neuroblastoma associated with a poor prognosis. In this subtype, MYC protein expression may be a new prognostic factor indicating more aggressive clinical behavior than MYCN amplification and subsequent MYCN protein expression.

journal_name

Cancer

journal_title

Cancer

authors

Wang LL,Suganuma R,Ikegaki N,Tang X,Naranjo A,McGrady P,London WB,Hogarty MD,Gastier-Foster JM,Look AT,Park JR,Maris JM,Cohn SL,Seeger RC,Shimada H

doi

10.1002/cncr.28251

subject

Has Abstract

pub_date

2013-10-15 00:00:00

pages

3718-26

issue

eissn

0008-543X

issn

1097-0142

journal_volume

119

pub_type

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Neuroblastoma of undifferentiated subtype, prognostic significance of prominent nucleolar formation, and MYC/MYCN protein expression: a report from the Children's Oncology Group.