PMID- 32790583
OWN - NLM
STAT- MEDLINE
DCOM- 20210906
LR  - 20210906
IS  - 1615-5742 (Electronic)
IS  - 1093-5266 (Linking)
VI  - 23
IP  - 6
DP  - 2020 Nov-Dec
TI  - Pediatric Soft Tissue Tumors With BCOR ITD Express EGFR but Not OLIG2.
PG  - 424-430
LID - 10.1177/1093526620945528 [doi]
AB  - INTRODUCTION: Somatic internal tandem duplication of 3' of BCOR (BCOR ITD) has 
      been found in clear cell sarcomas of the kidney (CCSK), soft tissue 
      undifferentiated round cell sarcomas/primitive myxoid mesenchymal tumors of 
      infancy (URCS/PMMTI), and a subgroup of central nervous system high-grade 
      neuroepithelial tumors (CNS-HGNET). BCOR ITD+ tumors share morphologic features. 
      Expression of OLIG2 and epidermal growth factor receptor (EGFR) has been reported 
      in CNS-HGNET with BCOR ITD. Here, we characterize OLIG2 and EGFR expression in 
      URCS/PMMTI with BCOR ITD. METHODS: Paraffin blocks of 9 polymerase chain 
      reaction-confirmed soft tissue BCOR ITD+ tumors (URCS/PMMTI) were 
      immunophenotyped for OLIG2 and EGFR expression and scored semiquantitatively by 
      percentage of positive cells and intensity of staining as negative, 1+, 2+, and 
      3+. Fluorescence in situ hybridization (FISH) for EGFR amplification was 
      performed (amplification EGFR/CEP7 ratio >/=2.0). RESULTS: All 9 tumors showed 
      membrane/cytoplasmic expression of EGFR, strong and diffuse (3+) in 8 cases; weak 
      (+2) in 1. FISH detected no EGFR amplification. OLIG2 was negative in all. 
      CONCLUSIONS: EGFR is overexpressed in pediatric URCS/PMMTI with BCOR ITD and may 
      be related to transcriptional upregulation of EGFR by BCOR ITD. OLIG2 negative 
      staining differentiates URCS/PMMTI from CNS-HGNET. This finding may further 
      support the possibility that these tumors have a different stem cell of origin.
FAU - Salgado, Claudia M
AU  - Salgado CM
AD  - Department of Pathology, UPMC Children's Hospital of Pittsburgh. University of 
      Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
FAU - Zin, Angelica
AU  - Zin A
AD  - Clinica di Oncoematologia Pediatrica Azienda Ospedaliera, Universita di Padova, 
      Padua, Italy.
FAU - Garrido, Marta
AU  - Garrido M
AD  - Unidad de Anatomia Patologica, Hospital Universitari Vall d'Hebron, Barcelona, 
      Spain.
FAU - Kletskaya, Irina
AU  - Kletskaya I
AUID- ORCID: 0000-0002-8552-7682
AD  - Russian Children's Clinical Hospital of Pirogov Russian National Research Medical 
      University of the Ministry of Healthcare, Russian Federation, Moscow, Russia.
FAU - DeVito, Rita
AU  - DeVito R
AD  - Ospedale Pediatrico Bambino Gesu, Istituto Ricovero e Cura a Carattere 
      Scientifico, Rome, Italy.
FAU - Reyes-Mugica, Miguel
AU  - Reyes-Mugica M
AUID- ORCID: 0000-0001-5698-3702
AD  - Department of Pathology, UPMC Children's Hospital of Pittsburgh. University of 
      Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
FAU - Bisogno, Gianni
AU  - Bisogno G
AD  - Dipartimento Salute della Donna e Del Bambino, Universita di Padova, Padua, 
      Italy.
FAU - Donofrio, Vittoria
AU  - Donofrio V
AD  - Pathology Unit Department of Pathology, Santobono-Pausilipon Children's Hospital, 
      Via Posillipo, Naples, Italy.
FAU - Alaggio, Rita
AU  - Alaggio R
AD  - Ospedale Pediatrico Bambino Gesu, Istituto Ricovero e Cura a Carattere 
      Scientifico, Rome, Italy.
LA  - eng
PT  - Journal Article
DEP - 20200813
PL  - United States
TA  - Pediatr Dev Pathol
JT  - Pediatric and developmental pathology : the official journal of the Society for 
      Pediatric Pathology and the Paediatric Pathology Society
JID - 9809673
RN  - 0 (BCOR protein, human)
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (OLIG2 protein, human)
RN  - 0 (Oligodendrocyte Transcription Factor 2)
RN  - 0 (Proto-Oncogene Proteins)
RN  - 0 (Repressor Proteins)
RN  - EC 2.7.10.1 (EGFR protein, human)
RN  - EC 2.7.10.1 (ErbB Receptors)
SB  - IM
MH  - Biomarkers, Tumor/genetics/*metabolism
MH  - Child, Preschool
MH  - ErbB Receptors/metabolism
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunohistochemistry
MH  - Immunophenotyping
MH  - In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Oligodendrocyte Transcription Factor 2/*metabolism
MH  - Proto-Oncogene Proteins/*genetics/metabolism
MH  - Repressor Proteins/*genetics/metabolism
MH  - Retrospective Studies
MH  - Sarcoma/genetics/*metabolism/pathology
MH  - Soft Tissue Neoplasms/genetics/*metabolism/pathology
MH  - *Tandem Repeat Sequences
MH  - Up-Regulation
OTO - NOTNLM
OT  - BCOR
OT  - BCOR ITD
OT  - EGFR
OT  - OLIG2
OT  - PMMTI
OT  - URCS
EDAT- 2020/08/14 06:00
MHDA- 2021/09/07 06:00
CRDT- 2020/08/14 06:00
PHST- 2020/08/14 06:00 [pubmed]
PHST- 2021/09/07 06:00 [medline]
PHST- 2020/08/14 06:00 [entrez]
AID - 10.1177/1093526620945528 [doi]
PST - ppublish
SO  - Pediatr Dev Pathol. 2020 Nov-Dec;23(6):424-430. doi: 10.1177/1093526620945528. 
      Epub 2020 Aug 13.