Soft Tissues: Ewings tumors/Primitive neurectodermal tumor with t(16;21)(p11;q22) FUS/ERG
2005-01-01 Jean-Loup Huret Affiliation1.Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, France
Clinics and Pathology
Note
t(16;21)(p11;q22) has been found in rare cases of Ewing tumours, a paediatric neoplasm with small round-cells derived from neural crests cells usually associated with translocations involving EWSR1
Cytogenetics
Prognosis
recent treatments have improved the prognosis of Ewings tumours. The prognosis is mainly determined by the presence of metastases at the time of diagnosis
Cytogenetics
Genes Involved and Proteins
Gene name
FUS (fusion involved in t (12;16) in malignant liposarcoma)
Location
16p11.2
Protein description
RNA binding protein; member of the TET family, like EWSR1
Gene name
ERG (v-ets erythroblastosis virus E26 oncogene like (avian))
Location
21q22.2
Protein description
ETS transcription factor
Result of the chromosomal anomaly
Description
5 FUS including exons 1 to 6, 7 or 8 - 3 ERG from exon 7, 8 or 9 to C-term.
Description
N-term FUS transactivation domain fused to the C-term DNA binding ETS domain of ERG
Oncogenesis
seems to act as a transcriptional activator
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 32433508 | 2020 | Gain of chromosome 21 in hematological malignancies: lessons from studying leukemia in children with Down syndrome. | 146 |
| 31850234 | 2019 | Donor-Derived CD123-Targeted CAR T Cell Serves as a RIC Regimen for Haploidentical Transplantation in a Patient With FUS-ERG+ AML. | 74 |
| 38798454 | 2024 | EZH2-driven immune evasion defines high-risk pediatric AML with t(16;21) FUS::ERG gene fusion. | 61 |
| 30150206 | 2018 | Prognostic impact of t(16;21)(p11;q22) and t(16;21)(q24;q22) in pediatric AML: a retrospective study by the I-BFM Study Group. | 60 |
| 22140532 | 2011 | ERG deregulation induces PIM1 over-expression and aneuploidy in prostate epithelial cells. | 55 |
| 26148230 | 2016 | The oncofusion protein FUS-ERG targets key hematopoietic regulators and modulates the all-trans retinoic acid signaling pathway in t(16;21) acute myeloid leukemia. | 54 |
| 36916780 | 2023 | RNA sequencing of myeloid sarcoma, shed light on myeloid sarcoma stratification. | 54 |
| 36789545 | 2023 | Pathologic, cytogenetic, and molecular features of acute myeloid leukemia with megakaryocytic differentiation: A report from the Children's Oncology Group. | 51 |
| 27286459 | 2016 | Effective molecular targeting of CDK4/6 and IGF-1R in a rare FUS-ERG fusion CDKN2A-deletion doxorubicin-resistant Ewing's sarcoma patient-derived orthotopic xenograft (PDOX) nude-mouse model. | 51 |
| 36181538 | 2022 | TLS/FUS-ERG fusion gene in acute leukemia and myelodysplastic syndrome evolved to acute leukemia: report of six cases and a literature review. | 49 |
| 29262551 | 2017 | The combination of temozolomide-irinotecan regresses a doxorubicin-resistant patient-derived orthotopic xenograft (PDOX) nude-mouse model of recurrent Ewing's sarcoma with a FUS-ERG fusion and CDKN2A deletion: Direction for third-line patient therapy. | 49 |
| 10523652 | 1999 | Dual transforming activities of the FUS (TLS)-ERG leukemia fusion protein conferred by two N-terminal domains of FUS (TLS). | 48 |
| 37660196 | 2023 | FUS-ERG induces late-onset azacitidine resistance in acute myeloid leukaemia cells. | 47 |
| 30895378 | 2019 | DNA methylation profiling distinguishes Ewing-like sarcoma with EWSR1-NFATc2 fusion from Ewing sarcoma. | 40 |
| 18648544 | 2008 | IGF1 is a common target gene of Ewing's sarcoma fusion proteins in mesenchymal progenitor cells. | 39 |
| 26690869 | 2016 | Ewing sarcoma with ERG gene rearrangements: A molecular study focusing on the prevalence of FUS-ERG and common pitfalls in detecting EWSR1-ERG fusions by FISH. | 36 |
| 33708041 | 2021 | Distribution of fusion transcripts and its clinical impact in patients with acute myeloid leukemia in Sudan. | 34 |
| 24068373 | 2013 | Cryptic FUS-ERG fusion identified by RNA-sequencing in childhood acute myeloid leukemia. | 32 |
| 32362012 | 2020 | The clinical heterogeneity of round cell sarcomas with EWSR1/FUS gene fusions: Impact of gene fusion type on clinical features and outcome. | 32 |
| 27033758 | 2016 | [Clinical characteristics of acute myeloid leukemia with t (16;21) (p11;q22):nine cases report and literature review]. | 30 |
| 38742238 | 2024 | The RTK-RAS signaling pathway is enriched in patients with rare acute myeloid leukemia harboring t(16;21)(p11;q22)/FUS::ERG. | 28 |
| 29225830 | 2017 | Acute basophilic leukemia associated with the t(16;21)(p11;q22)/FUS-ERG fusion gene. | 28 |
| 39857993 | 2025 | PROTAC-Mediated GSPT1 Degradation Impairs the Expression of Fusion Genes in Acute Myeloid Leukemia. | 28 |
| 17620387 | 2007 | Ewing sarcoma with novel translocation t(2;16) producing an in-frame fusion of FUS and FEV. | 19 |
| 31788268 | 2019 | Salvage therapy with azacitidine for pediatric acute myeloid leukemia with t(16;21)(p11;q22)/FUS-ERG and early relapse after allogeneic blood stem cell transplantation: A case report. | 18 |
| 26770812 | 2016 | FUS-ERG gene fusion in isolated myeloid sarcoma showing uncommon clinical features. | 10 |
| 25830133 | 2015 | A case of pediatric ALL with t(16;21)(p11.2;q22) and FUS-ERG rearrangement. | 9 |
| 33868529 | 2021 | Radiological and pathological diagnosis of an incidental Askin tumor. | 9 |
| 33442361 | 2020 | Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma. | 6 |
| 35738886 | 2022 | Gene of the month: ERG. | 0 |
| 38651500 | 2024 | Identification of the characteristics and prognostic impact of FUS::ERG and RUNX1::CBFA2T3 fusion genes in adult acute myeloid leukemia patients. | 0 |
| 29427188 | 2018 | Molecular characteristic of acute leukemias with t(16;21)/FUS-ERG. | 0 |
| 7970732 | 1994 | TLS/FUS fusion domain of TLS/FUS-erg chimeric protein resulting from the t(16;21) chromosomal translocation in human myeloid leukemia functions as a transcriptional activation domain. | 0 |
| 35503261 | 2022 | A novel HNRNPH1::ERG rearrangement in aggressive acute myeloid leukemia. | 0 |
| 38639201 | 2024 | Immunophenotypic clustering in paediatric acute myeloid leukaemia. | 0 |
| 37828859 | 2023 | Dissolving Fusion Oncoprotein Condensates to Reverse Aberrant Gene Expression. | 0 |
| 9315104 | 1997 | Characteristic sequence motifs at the breakpoints of the hybrid genes FUS/CHOP, EWS/CHOP and FUS/ERG in myxoid liposarcoma and acute myeloid leukemia. | 0 |
| 12907633 | 2003 | FUS/ERG gene fusions in Ewing's tumors. | 0 |
| 9242552 | 1997 | Consistent detection of TLS/FUS-ERG chimeric transcripts in acute myeloid leukemia with t(16;21)(p11;q22) and identification of a novel transcript. | 0 |
| 7533529 | 1994 | Fusion of the FUS gene with ERG in acute myeloid leukemia with t(16;21)(p11;q22). | 0 |
| 39658629 | 2025 | Allogeneic hematopoietic stem cell transplantation in adult acute myeloid leukemia with t(16;21)(p11;q22)/FUS::ERG. | 0 |
| 38953149 | 2024 | High DOCK1 expression identifies a distinct prognostic subgroup of pediatric acute myeloid leukemia: Results of the Japanese Pediatric Leukemia/Lymphoma Study Group AML-05 trial. | 0 |
| 16263589 | 2005 | TLS/FUS-ERG fusion gene in acute lymphoblastic leukemia with t(16;21)(p11;q22) and monitoring of minimal residual disease. | 0 |
| 27183148 | 2016 | A t(16;21)(p11;q22) in Acute Myeloid Leukemia (AML) Resulting in Fusion of the FUS/TLS and ERG Genes: A Review of the Literature. | 0 |
| 33894645 | 2021 | Rare and favorable prognosis of pediatric acute lymphoblastic leukemia with TLS-ERG fusion gene: Case report with long-term follow-up and review of literature. | 0 |
| 19781443 | 2009 | Detection of FUS-ERG chimeric transcript in two cases of acute myeloid leukemia with t(16;21)(p11.2;q22) with unusual characteristics. | 0 |
| 34747554 | 2022 | Endobronchial ultrasound-guided fine needle aspiration diagnosis of metastatic FUS-ERG-rearranged extraskeletal Ewing sarcoma following 15 years of untreated metastatic recurrence. | 0 |
| 8187069 | 1994 | An RNA-binding protein gene, TLS/FUS, is fused to ERG in human myeloid leukemia with t(16;21) chromosomal translocation. | 0 |
| 16737920 | 2006 | Unusual type of TLS/FUS-ERG chimeric transcript in a pediatric acute myelocytic leukemia with 47,XX,+10,t(16;21)(p11;q22). | 0 |
| 19737655 | 2009 | Ewing sarcoma-peripheral neuroectodermal tumor of the kidney with a FUS-ERG fusion transcript. | 0 |
| 36448876 | 2023 | UBTF-internal tandem duplication as a novel poor prognostic factor in pediatric acute myeloid leukemia. | 0 |
| 28766906 | 2017 | CD66c (KOR-SA3544) antigen expression of leukemic blasts in pediatric acute myeloid leukemia with TLS/FUS-ERG fusion transcript. | 0 |
| 39522640 | 2025 | Superficial Neurocristic FET::ETS Fusion Tumor: Expanding the Clinicopathological and Molecular Genetic Spectrum of a Recently Described Entity. | 0 |
| 29959436 | 2019 | Allogeneic hematopoietic stem cell transplantation for children and adolescents with high-risk cytogenetic AML: distinctly poor outcomes of FUS-ERG-positive cases. | 0 |
| 28681998 | 2018 | Eribulin regresses a doxorubicin-resistant Ewing's sarcoma with a FUS-ERG fusion and CDKN2A-deletion in a patient-derived orthotopic xenograft (PDOX) nude mouse model. | 0 |
| 10655065 | 2000 | Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death. | 0 |
| 20620604 | 2010 | Two childhood cases of acute leukemia with t(16;21)(p11.2;q22): second case report of infantile acute lymphoblastic leukemia with unusual type of FUS-ERG chimeric transcript. | 0 |
| 30277115 | 2019 | Panoramic view of common fusion genes in a large cohort of Chinese de novo acute myeloid leukemia patients. | 0 |
| 33186348 | 2020 | Acute myeloid leukemia associated with t(16:21)(p11;q22) in a pediatric patient. | 0 |
| 23210573 | 2013 | Detection and clinical significance of gene rearrangements in Chinese patients with adult acute lymphoblastic leukemia. | 0 |
| 20694842 | 2010 | CD56 antigen expression and hemophagocytosis of leukemic cells in acute myeloid leukemia with t(16;21)(p11;q22). | 0 |
| 21536242 | 2011 | Establishment and characterization of a novel acute myeloid leukemia cell line, JIH-4, carrying a t(16;21)(p11.2;q22) and expressing the FUS-ERG fusion. | 0 |
| 29067751 | 2018 | Monitoring of fusion gene transcripts to predict relapse in pediatric acute myeloid leukemia. | 0 |
| 9178886 | 1997 | Inhibition of apoptosis by normal and aberrant Fli-1 and erg proteins involved in human solid tumors and leukemias. | 0 |
| 10354136 | 1999 | Myeloid differentiation antigen and cytokine receptor expression on acute myelocytic leukaemia cells with t(16;21)(p11;q22): frequent expression of CD56 and interleukin-2 receptor alpha chain. | 0 |
| 24374719 | 2014 | RUNX1 mutation associated with clonal evolution in relapsed pediatric acute myeloid leukemia with t(16;21)(p11;q22). | 0 |
| 11505530 | 2001 | [AML(M7) associated with t(16;21)(p11;q22) showing relapse after unrelated bone marrow transplantation and disappearance of TLS/FUS-ERG mRNA]. | 0 |
| 9096702 | 1997 | Establishment of a novel human acute myeloblastic leukemia cell line (YNH-1) with t(16;21), t(1;16) and 12q13 translocations. | 0 |
| 11340253 | 2001 | Detection of minimal residual disease in a patient having acute myelogenous leukemia with t(16;21)(p11;q22) treated by allogeneic bone marrow transplantation. | 0 |
| 19893346 | 2009 | [Two cases of acute myeloid leukemia with t(16;21)(p11;q22) and TLS/FUS-ERG fusion transcripts]. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 2357684 | 1990 | Acute nonlymphocytic leukemia with t(16;21). | Berkowicz M et al |
| 12091356 | 2002 | Cytogenetic profile of childhood and adult megakaryoblastic leukemia (M7): a study of the Groupe Français de Cytogénétique Hématologique (GFCH). | Dastugue N et al |
| 1606569 | 1992 | t(16;21) in a Ph positive CML. | Ferro MR et al |
| 9549830 | 1997 | Detection of minimal residual disease in cerebro-spinal fluid of a patient with acute myelogenous leukemia with t(16;21)(p11;q22) translocation by reverse transcriptase-polymerase chain reaction. | Harigae H et al |
| 14654949 | 2004 | Breakpoint differentiation in chromosomal aberrations of hematological malignancies: Identification of 33 previously unrecorded breakpoints. | Heller A et al |
| 8719898 | 1995 | Acute non-lymphoblastic leukaemia with t(16;21): case report with a review of the literature. | Hiyoshi M et al |
| 7794765 | 1995 | Establishment and characterization of IRTA17 and IRTA21, two novel acute non-lymphocytic leukaemia cell lines with t(16;21) translocation. | Hiyoshi M et al |
| 10446493 | 1999 | Hemophagocytosis by leukemic blasts in a case of acute megakaryoblastic leukemia with t(16;21)(p11;q22). | Imashuku S et al |
| 9242552 | 1997 | Consistent detection of TLS/FUS-ERG chimeric transcripts in acute myeloid leukemia with t(16;21)(p11;q22) and identification of a novel transcript. | Kong XT et al |
| 2065316 | 1991 | Translocation (16;21)(p11;q22) in acute monoblastic leukemia with erythrophagocytosis. | Marosi C et al |
| 8453103 | 1993 | The 8;21 chromosome translocation in acute myeloid leukemia is always detectable by molecular analysis using AML1. | Maseki N et al |
| 3860282 | 1985 | Acute nonlymphoblastic leukemia with bone marrow eosinophilia and structural anomaly of chromosome 16. | Mecucci C et al |
| 3164243 | 1988 | Translocation (8;21) and its variants in acute nonlymphocytic leukemia. The relative importance of chromosomes 8 and 21 to the genesis of the disease. | Minamihisamatsu M et al |
| 2036642 | 1991 | t(16;21)(p11.2;q22): a recurrent primary rearrangement in ANLL. | Morgan R et al |
| 8242597 | 1993 | Acute non-lymphocytic leukemia with t(16;21). | Nobbs MC et al |
| 8039166 | 1994 | Translocation (16;21)(p11;q22) in acute nonlymphocytic leukemia. | Okada K et al |
| 10634649 | 2000 | Acute myeloid leukemia possessing jumping translocation is related to highly elevated levels of EAT/mcl-1, a Bcl-2 related gene with anti-apoptotic functions. | Okita H et al |
| 7533529 | 1994 | Fusion of the FUS gene with ERG in acute myeloid leukemia with t(16;21)(p11;q22). | Panagopoulos I et al |
| 10572083 | 1999 | Chromosomal abnormalities in 478 children with acute myeloid leukemia: clinical characteristics and treatment outcome in a cooperative pediatric oncology group study-POG 8821. | Raimondi SC et al |
| 2125791 | 1990 | 16;21 translocation in acute nonlymphocytic leukemia with abnormal eosinophils: a unique subtype. | Sadamori N et al |
| 8139114 | 1994 | [Acute monoblastic leukemia (M5a) with dysmegakaryocytopoiesis associated with t(16;21) (p11;q22)]. | Satoh K et al |
| 7517211 | 1994 | HLA-DR-, CD33+, CD56+, CD16- myeloid/natural killer cell acute leukemia: a previously unrecognized form of acute leukemia potentially misdiagnosed as French-American-British acute myeloid leukemia-M3. | Scott AA et al |
| 10354136 | 1999 | Myeloid differentiation antigen and cytokine receptor expression on acute myelocytic leukaemia cells with t(16;21)(p11;q22): frequent expression of CD56 and interleukin-2 receptor alpha chain. | Shikami M et al |
| 12907633 | 2003 | FUS/ERG gene fusions in Ewing's tumors. | Shing DC et al |
| 9592183 | 1998 | Cytogenetic analysis of de novo acute myeloid leukemia with trilineage myelodysplasia in comparison with myelodysplastic syndrome evolving to acute myeloid leukemia. | Tamura S et al |
Citation
Jean-Loup Huret
Soft Tissues: Ewings tumors/Primitive neurectodermal tumor with t(16;21)(p11;q22) FUS/ERG
Atlas Genet Cytogenet Oncol Haematol. 2005-01-01
Online version: http://atlasgeneticsoncology.org/solid-tumor/5329/soft-tissues-ewings-tumors-primitive-neurectodermal-tumor-with-t(16;21)(p11;q22)-fus-erg
Historical Card
1998-02-01 Soft Tissues: Ewings tumors/Primitive neurectodermal tumor with t(16;21)(p11;q22) FUS/ERG by Christine Pérot Affiliation
