PMID- 36865795
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230304
IS  - 2234-943X (Print)
IS  - 2234-943X (Electronic)
IS  - 2234-943X (Linking)
VI  - 13
DP  - 2023
TI  - Segmental chromosomal aberrations as the poor prognostic factor in children over 
      18 months with stage 3 neuroblastoma without MYCN amplification.
PG  - 1134772
LID - 10.3389/fonc.2023.1134772 [doi]
LID - 1134772
AB  - INTRODUCTION: Patients with stage 3 neuroblastoma (NBL) according to 
      International Neuroblastoma Staging System (INSS) without MYCN amplification 
      represent a heterogenous group with respect to disease presentation and 
      prognosis. METHODS: Retrospective analysis of 40 stage 3 patients with NBL 
      without MYCN amplification was performed. The prognostic value of age at 
      diagnosis (under 18 vs over 18 months), International Neuroblastoma Pathology 
      Classification (INPC) diagnostic category and presence of segmental or numerical 
      chromosomes aberrations were evaluated, as well as biochemical markers. Array 
      comparative genomic hybridization (aCGH) for analyzing copy number variations and 
      Sanger sequencing for ALK point mutations were done. RESULTS: In 12 patients (two 
      patients under 18 months), segmental chromosomal aberrations (SCA) were found and 
      numerical chromosomal aberrations (NCA) were found in 16 patients (14 patients 
      under 18 months). In children over 18 months SCA were more common (p=0.0001). 
      Unfavorable pathology was significantly correlated with SCA genomic profile 
      (p=0.04) and age over 18 months (p=0.008). No therapy failures occurred in 
      children with NCA profile over or under 18 months or in children under 18 months, 
      irrespective of pathology and CGH results. Three treatment failures occurred in 
      the SCA group, in one patient CGH profile was not available. For the whole group 
      at 3, 5 and 10-year OS and DFS were 0.95 (95% CI 0.81-0.99), 0.91 (95% CI 
      0.77-0.97) and 0.91 (95% CI 0.77-0.97), and 0.95 (95% CI 0.90-0.99), 0.92 (95% CI 
      0.85-0.98) and 0.86 (95% CI 0.78-0.97), respectively. DFS was significantly lower 
      in the SCA group than in the NCA group (3-years, 5-years, and 10-years DFS 0.92 
      (95% CI 0.53-0.95), 0.80 (95% CI 0.40-0.95) and 0.60 (95% CI 0.16-0.87) vs 1.0, 
      1.0 and 1.0, respectively, p=0.005). CONCLUSIONS: The risk of treatment failure 
      was higher in patients with SCA profile, but only in patients over 18 months. All 
      relapses occurred in children having obtained the complete remission, with no 
      previous radiotherapy. In patients over 18 months, SCA profile should be taken 
      into consideration for therapy stratification as it increases the risk of relapse 
      and this group may require more intensive treatment.
CI  - Copyright (c) 2023 Wieczorek, Szewczyk, Klekawka, Stefanowicz, Ussowicz, Drabik, 
      Pawinska-Wasikowska and Balwierz.
FAU - Wieczorek, Aleksandra
AU  - Wieczorek A
AD  - Department of Pediatric Oncology and Hematology, Medical College, Jagiellonian 
      University, Krakow, Poland.
AD  - Department of Pediatric Oncology and Hematology, University Children's Hospital 
      of Krakow, Krakow, Poland.
FAU - Szewczyk, Katarzyna
AU  - Szewczyk K
AD  - Department of Medical Genetics, Institute of Pediatrics, Medical College, 
      Jagiellonian University, Krakow, Poland.
FAU - Klekawka, Tomasz
AU  - Klekawka T
AD  - Department of Pediatric Oncology and Hematology, University Children's Hospital 
      of Krakow, Krakow, Poland.
FAU - Stefanowicz, Joanna
AU  - Stefanowicz J
AD  - Department of Pediatrics, Pediatric Hematology and Oncology, Medical University 
      of Gdansk, Gdansk, Poland.
FAU - Ussowicz, Marek
AU  - Ussowicz M
AD  - Department of Pediatric Bone Marrow Transplantation, Oncology, and Hematology, 
      Wroclaw Medical University, Wroclaw, Poland.
FAU - Drabik, Grazyna
AU  - Drabik G
AD  - Department of Pathology, University Children's Hospital of Krakow, Krakow, 
      Poland.
FAU - Pawinska-Wasikowska, Katarzyna
AU  - Pawinska-Wasikowska K
AD  - Department of Pediatric Oncology and Hematology, Medical College, Jagiellonian 
      University, Krakow, Poland.
AD  - Department of Pediatric Oncology and Hematology, University Children's Hospital 
      of Krakow, Krakow, Poland.
FAU - Balwierz, Walentyna
AU  - Balwierz W
AD  - Department of Pediatric Oncology and Hematology, Medical College, Jagiellonian 
      University, Krakow, Poland.
AD  - Department of Pediatric Oncology and Hematology, University Children's Hospital 
      of Krakow, Krakow, Poland.
LA  - eng
PT  - Journal Article
DEP - 20230214
PL  - Switzerland
TA  - Front Oncol
JT  - Frontiers in oncology
JID - 101568867
PMC - PMC9972431
OTO - NOTNLM
OT  - MYCN non-amplified neuroblastoma
OT  - neuroblastoma
OT  - prognostic factor
OT  - segmental chromosomal aberrations
OT  - stage 3
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/03/04 06:00
MHDA- 2023/03/04 06:01
PMCR- 2023/01/01
CRDT- 2023/03/03 02:37
PHST- 2022/12/30 00:00 [received]
PHST- 2023/01/30 00:00 [accepted]
PHST- 2023/03/03 02:37 [entrez]
PHST- 2023/03/04 06:00 [pubmed]
PHST- 2023/03/04 06:01 [medline]
PHST- 2023/01/01 00:00 [pmc-release]
AID - 10.3389/fonc.2023.1134772 [doi]
PST - epublish
SO  - Front Oncol. 2023 Feb 14;13:1134772. doi: 10.3389/fonc.2023.1134772. eCollection 
      2023.