High-Throughput Hybridization Assay as First-Line Diagnostic Test for Sarcomas: Clinical Assessment in a Tertiary Referral Center.

Context.--Sarcomas are rare and highly heterogeneous mesenchymal tumors with deceptive morphologic features that pose a challenge for precise diagnostics. Chromosomal rearrangements generating pathognomonic gene fusions are useful diagnostic markers, traditionally tested using single-plex standard o...

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Publicado en:Archives of Pathology & Laboratory Medicine Vol. 149; no. 6; pp. 511 - 519
Autores principales: Salguero-Aranda, Carmen, Perez, Marco, Vargas-Padilla, María Victoria, Beltrán-Povea, Amparo, Delgado-Bellido, Daniel, Marcilla, David, Civantos, Gema, de Álava, Enrique, Díaz-Martín, Juan
Formato: research tables/charts Journal Article
Publicado: College of American Pathologists Jun2025
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Jun2025
      vid: 149
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      pub: College of American Pathologists
      place: Northfield, Illinois
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        10.5858/arpa.2024-0202-OA
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        atl: High-Throughput Hybridization Assay as First-Line Diagnostic Test for Sarcomas: Clinical Assessment in a Tertiary Referral Center.
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        au:
          Salguero-Aranda, Carmen
          Perez, Marco
          Vargas-Padilla, María Victoria
          Beltrán-Povea, Amparo
          Delgado-Bellido, Daniel
          Marcilla, David
          Civantos, Gema
          de Álava, Enrique
          Díaz-Martín, Juan
        affil: Department of Pathology, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío, CSIC-Universidad de Sevilla, Seville, Spain
      sug:
        subj:
          Tumor Markers, Biological Blood
          Genes Physiology
          High-Throughput Screening Assays Methods
          Sarcoma Diagnosis
          Sarcoma Familial and Genetic
          Sensitivity and Specificity Evaluation
          Tertiary Health Care
          Referral and Consultation
          Sarcoma Therapy
          Human
          Retrospective Design
          Record Review
          Validation Studies
          Genes
          Prospective Studies
          Gene Expression Profiling
          Neoplasm Grading
          Oncologic Care Standards
          Carrier Proteins Physiology
          Signal Transduction Physiology
          Transcription Factors Physiology
          Cyclic AMP Metabolism
          Descriptive Statistics
          Data Analysis Software
      ab: Context.--Sarcomas are rare and highly heterogeneous mesenchymal tumors with deceptive morphologic features that pose a challenge for precise diagnostics. Chromosomal rearrangements generating pathognomonic gene fusions are useful diagnostic markers, traditionally tested using single-plex standard of care assays with limited diagnostic yield. NanoString nCounter technology has emerged as a robust solution with multiplexing capabilities. Objective.--To optimize NanoString effective coverage of specific entities and conduct a validation study to support its clinical implementation. Design.--We reconfigured a NanoString codeset by including a set of probes for detecting gene fusion variants of solitary fibrous tumors, low-grade fibromyxoid sarcomas/sclerosing epithelioid fibrosarcomas, and undifferentiated small round cell sarcomas, totaling 188 probes. A technical validation study was conducted with 96 retrospective samples. Additionally, 76 prospective samples were evaluated to assess the assay's clinical performance. Results.--Both technical and clinical validation studies showed that NanoString's codeset reached >88% sensitivity and 100% specificity, compared with standard of care methods, and superior diagnostic yield as a first-line test. Our design enabled the detection of almost all fusion variants of NGFI-A binding protein 2 (NAB2) with signal transducer and activator of transcription 6 (STAT6) in solitary fibrous tumors, as well as cAMP responsive element binding protein 3 like 1/2 (CREB3L1/2) rearrangements in all low-grade fibromyxoid sarcoma/sclerosing epithelioid fibrosarcoma cases. Identification of specific gene fusions of undifferentiated small round cell sarcoma was also improved, but additional strategies are necessary to attain full coverage. Conclusions.--The NanoString platform demonstrated good sensitivity, specificity, and superior diagnostic yield. It is a cost-effective assay with rapid turnaround time, low sample consumption, streamlined analysis, and easy customization. Therefore, it is a promising alternative first-line diagnostic tool for routine sarcoma testing.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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