EWS _ EWSR1
- Known as:
- EWS _ EWSR1
- Catalog number:
- Y214147
- Product Quantity:
- 200ul
- Category:
- -
- Supplier:
- ABM
- Gene target:
- EWS _ EWSR1
Related genes to: EWS _ EWSR1
- Gene:
- EWSR1 NIH gene
- Name:
- EWS RNA binding protein 1
- Previous symbol:
- -
- Synonyms:
- EWS
- Chromosome:
- 22q12.2
- Locus Type:
- gene with protein product
- Date approved:
- 1992-11-27
- Date modifiied:
- 2016-04-25
Related products to: EWS _ EWSR1
Related articles to: EWS _ EWSR1
- We present a 25-year-old female with a two-year history of clear cell sarcoma (CCS) of the hand. The patient presented with a painless left hypothenar tumor without neurological or functional compromise. MRI was compatible with sarcoma (4.8x3.4x4.9cm) and incisional biopsy confirmed CCS by immunohistochemistry (MITF1 and EWSR1 rearrangement both positive). The patient received preoperative radiotherapy for tumor reduction (approximately 80%). A wide resection was performed with negative margins of 2 cm, including third-fifth metacarpals, partial carpectomy and amputation of the fifth finger due to intraoperative ischemia. The lesion was reconstructed with a free fibula flap whose survival and postoperative short-term evolution were favorable. Six months after surgery, the patient presented partial mobility under rehabilitation with no evidence of recurrence or metastasis. Disabilities of the Arm, Shoulder and Hand (DASH), Musculoskeletal Tumor Society (MSTS) score, grip and pressure strength could not be determined. Our case is very particular due to the hypothenar involvement with central multistructural involvement and an atypical slow evolution. The aggressive therapeutic approach with microsurgical resection, reconstruction and functional preservation using a multisegmented fibula free osteocutaneous flap was favorable for the patient's outcome. - Source: PubMed
Publication date: 2026/07/23
Gonzalez-Martinez Cesar AGutierrez-Gonzalez Jorge ASalas-Trevino DanielGonzález-Cantú Cynthia MCastro-Govea Yanko - Targeting transcription factor fusion-positive cancers has proven challenging for decades. In SARC037, Grohar et al. show that histology-specific dosing of trabectedin and low-dose irinotecan is active in advanced Ewing sarcoma and downregulates EWSR1::FLI1 activity. - Source: PubMed
Publication date: 2026/08/21
Shulman David SJaneway Katherine A - Ewing sarcoma breakpoint region 1 ():: Fifth Ewing variant ()-positive acute leukemia is exceptionally rare, and its clinicopathological features, molecular profile, and optimal treatment remain poorly defined. Here, we report a case of acute myeloid leukemia (AML) with bone marrow hypocellularity, aberrant lymphoid antigen expression, and an fusion. A 45-year-old man was admitted with a 1-month history of petechiae and fatigue. Complete blood count (CBC) showed pancytopenia. Bone marrow (BM) examination revealed hypocellular marrow, with blasts accounting for 62% of nucleated cells. Flow cytometry (FCM) revealed a predominantly myeloid immunophenotype accompanied by aberrant expression of lymphoid-associated antigens. Conventional karyotyping did not identify a characteristic balanced translocation. Targeted next-generation sequencing (NGS) detected concurrent and mutations. RNA sequencing (RNA-seq) of the bone marrow sample identified the fusion. Based on these findings, the patient was diagnosed with de novo AML with bone marrow hypocellularity and aberrant lymphoid antigen expression. Given the hypocellular marrow, venetoclax plus azacitidine (VA) was selected as induction therapy. The patient achieved morphological complete remission after one cycle, accompanied by a marked reduction in transcript levels, and remained in morphological remission during approximately 6 months of follow-up after subsequent homoharringtonine combined with venetoclax plus azacitidine (HVA) consolidation. Together with previously reported cases, this case further supports the notion that -positive acute leukemia may represent a distinct molecular subset characterized by lineage ambiguity, fusion-driven biology, and potentially unique therapeutic vulnerabilities. This case also suggests potential activity of venetoclax-based therapy in -positive leukemia and warrants further investigation in additional cases. - Source: PubMed
Publication date: 2026/08/19
Xia WenxiaZou JunyanChen XiaoyongLuo JingFeng ChunDai MaoDing YanhongZhou JihaoKe PengLi HuijunHou Junjie - Inflammatory and nested testicular sex cord tumor (INTSCT) is a recently recognized malignant testicular neoplasm characterized by distinctive morphologic, immunophenotypic, and molecular features, including frequent EWSR1::ATF1 gene fusions. Although prior series have established INTSCT as a distinct entity with aggressive behavior, its full clinicopathologic and molecular spectrum remains incompletely defined. Thirteen previously unreported INTSCT from multiple institutions were analyzed. Clinicopathologic features were reviewed, including clinical presentation, outcome, histologic findings, and immunophenotype. Molecular characterization was performed using RNA sequencing and/or fluorescence in situ hybridization (FISH) for EWSR1 and ATF1 rearrangements. Patients ranged from 22 to 71 years old (median 37). All presented with testicular masses. Among 8 patients with adequate follow-up, 7 developed metastases to retroperitoneal lymph nodes and 2 to lungs. Histologically, tumors consistently exhibited nested architecture, intratumoral and/or peritumoral inflammation, and thick collagenous stroma, with frequent cellular discohesion and vacuolated cytoplasm; one case showed a Leydig cell-like phenotype. Immunohistochemistry demonstrated high rates of inhibin-α, CD30, keratin, EMA, and SF1 expression, with absence of germ cell markers. Molecular studies revealed EWSR1::ATF1 fusion or EWSR1 rearrangement in 12 of 13 tumors. This multi-institutional series corroborates and expands prior observations, confirming INTSCT as a distinctive, aggressive malignant sex cord-stromal tumor with a highly recurrent EWSR1::ATF1 fusion. Awareness of its morphologic spectrum, including features mimicking Leydig cell tumor, is essential to avoid misdiagnosis and ensure appropriate clinical management. - Source: PubMed
Publication date: 2026/08/19
Acosta Andres MMichalova KvetoslavaBerney Daniel MKao Chia-SuiSangoi Ankur RWilliamson Sean RAlaghehbandan RezaIdrees Muhammad TDonnell Marie O'Okunade AdenikeColecchia MaurizioTenace NazarioMartinek PetrMichal MichalUlbright Thomas M - Ewing sarcoma is a rare, aggressive malignancy of small round blue cells that typically arises from bone or soft tissue. Primary intracranial involvement is exceedingly uncommon, particularly in adults, and is associated with diagnostic challenges, high recurrence rates, and poor prognosis. Limited data exist regarding optimal management strategies for recurrent intracranial Ewing sarcoma in adult patients. - Source: PubMed
Publication date: 2026/08/14
Imran JunaidRehman Sadiq UrOsama MuhammadKalsoom HaniaThada Pawan Kumar