|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
These results are in keeping with a "BCOR-alteration family" of renal and extrarenal neoplasms which includes CCSK and undifferentiated round cell sarcomas of soft tissue/primitive myxoid mesenchymal tumor of infancy (which typically harbor BCOR internal tandem duplication), and BCOR-CCNB3 sarcomas, all of which are primarily driven by BCOR overexpression and have overlapping (but not identical) clinicopathologic features.
|
28817404 |
2017 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
BCOR ITD were present in 1 uterine sarcoma diagnosed as HG-ESS and 2 undifferentiated sarcomas with uniform nuclear features, all of which lacked any of the recurrent chromosome translocations known to occur in ESS.
|
29200103 |
2018 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Accumulating evidence suggests that internal tandem duplications (ITD) of BCOR are oncogenic drivers in a subset of pediatric sarcomas and rare adult tumors.
|
30380541 |
2019 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
The spectrum of ELS is now expanding, and additional gene fusion partners besides DUX4 or CCNB3 have been detected, and the terms CIC or BCOR-rearranged sarcomas have recently been proposed.
|
27306060 |
2016 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
Nonetheless, NKX2-2 may be helpful to distinguish Ewing sarcoma from some histologic mimics including CIC-DUX4 and BCOR-CCNB3 sarcomas.
|
26847175 |
2016 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
Our results suggest that BCOR and/or an ECM-like protein could be involved in the pathogenesis of a subgroup of PNET or PNET-like sarcomas.
|
16133367 |
2005 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
The BCOR-CCNB3 positive sarcoma is a recently identified sarcoma morphologically and clinically similar to Ewing sarcoma in adolescents and young adults.
|
30064235 |
2019 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
In addition, we included related pathologic entities such as 8 CCSKs and other sarcomas with BCOR gene fusions.
|
27428733 |
2016 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
BCOR-CCNB3 sarcomas showed swirling fascicular growth.
|
31647130 |
2020 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Unsupervised clustering by RNAseq data revealed that tumors with BCOR genetic alterations, including BCOR-CCNB3, BCOR-MAML3, and BCOR ITD, formed a tight genomic group distinct from ES and CIC-rearranged sarcomas.
|
29300189 |
2018 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
Fine-Needle Aspiration Features of BCOR-CCNB3 Sarcoma.
|
31679010 |
2020 |
|
Malignant neoplasm of soft tissue
|
0.100 |
AlteredExpression
|
group |
BEFREE |
Finally, we show that CCNB3 immunohistochemistry is a powerful diagnostic marker for this subgroup of sarcoma and that overexpression of BCOR-CCNB3 or of truncated CCNB3 activates S phase in NIH3T3 cells.
|
22387997 |
2012 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
The discovery of recurrent BCOR ITDs defines a major oncogenic event in this childhood sarcoma with significant implications for diagnostic and therapeutic approaches to this tumour.
|
26573325 |
2015 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
A new group of sarcomas with a recurrent BCOR-CCNB3 gene fusion has been recently identified in previously unclassifiable small round cell sarcomas.
|
28756981 |
2017 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities.
|
24805859 |
2014 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
Sarcomas harbouring BCOR-CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum.
|
27228320 |
2016 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
In the control cohort, BCOR ITD was found only in 3 CCSK cases but not in the other sarcomas.
|
26945340 |
2016 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
Heterogeneous elements included a myxoid spindle cell component in three BCOR-CCNB3 sarcomas and an epithelioid cell component in two CIC-associated sarcomas (one CIC-DUX4-positive and one CIC-DUX4-negative sarcomas).
|
28197724 |
2017 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
These include gene fusions in vascular neoplasms (FOSB, CAMTA1 and TFE3), round cell sarcomas (BCOR, DUX4 and WT1), and fibroblastic/myofibroblastic tumors (STAT6, ALK and Pan-TRK); amplifications in well-differentiated and dedifferentiated liposarcomas (MDM2 and CDK4); and deletions in several aggressive neoplasms (SMARCB1 and SMARCA4).
|
30382607 |
2019 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
NKX2.2, ETV4 and BCOR immunoreactivity was observed in all ES, CIC-rearranged sarcomas and sarcomas with BCOR alteration, respectively.
|
29661713 |
2018 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
RNA-sequencing data identified one <i>BCOR-CCNB3</i> gene fusion-positive sarcoma occurring in the sacrum of a 17-year-old male patient.
|
31632552 |
2019 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
In this review we discuss the main categories of undifferentiated round cell sarcoma, in relation to Ewing sarcoma and its molecular variants, with particular emphasis on the genetic and biologic features of recently described entities including desmoplastic small round cell tumor and CIC-DUX4 as well as BCOR-CCNB3-associated round cell sarcomas.
|
24801613 |
2014 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Five additional undifferentiated sarcomas with BCOR-CCNB3 fusions were identified in a series of 42 pediatric and adult unclassified sarcomas.
|
25360585 |
2015 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
More than 50% of cases stained positive for SATB2 and Pax8, raising the hypothesis of a potential use of these markers in the identification of BCOR-CCNB3 positive undifferentiated/unclassified sarcomas.
|
28420319 |
2019 |
|
Malignant neoplasm of soft tissue
|
0.100 |
Biomarker
|
group |
BEFREE |
The tumor showed an undifferentiated phenotype, including negativity for cytokeratin, although it was immunoreactive to BCOR and MUC4, and was initially suspected as BCOR-associated sarcoma.
|
31385070 |
2020 |