YAP1-TFE3 epithelioid hemangioendothelioma: a case without vasoformation and a new transcript variant.

Epithelioid hemangioendothelioma is a rare vascular tumor of borderline malignancy characterized by recurrent WWTR1-CAMTA1 gene fusions in approximately 90 % of cases. In addition, a recurrent YAP1-TFE3 gene fusion has been identified in WWTR1-CAMTA1 negative epithelioid hemangioendotheliomas. This...

Descripción completa

Detalles Bibliográficos
Publicado en:Virchows Archiv: European Journal of Pathology Vol. 466; no. 4; pp. 473 - 479
Autores principales: Puls, Florian, Niblett, Angela, Clarke, Jade, Kindblom, Lars-Gunnar, McCulloch, Tom
Formato: case study Journal Article
Publicado: Springer Nature Apr2015
Acceso en línea:Ver este registro en EBSCOhost
fields @attributes:
  recordID: 1
pdfLink:
plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109780942&site=ehost-live
header:
  @attributes:
    shortDbName: ccm
    uiTerm: 109780942
    longDbName: CINAHL Complete
    uiTag: AN
  controlInfo:
    bkinfo:
    dissinfo:
    jinfo:
      jid:
        09456317
        O1Z
      jtl: Virchows Archiv: European Journal of Pathology
      issn: 09456317
      maglogo: N
    pubinfo:
      dt: Apr2015
      vid: 466
      iid: 4
      pid: 237
      pub: Springer Nature
      place: New York, New York
    artinfo:
      ui:
        109780942
        NLM25680571
        2012969232
        10.1007/s00428-015-1730-y
        NLM25680571
        109780942
      ppf: 473
      ppct: 6
      formats:
        fmt:
          @attributes:
            type: P
      tig:
        atl: YAP1-TFE3 epithelioid hemangioendothelioma: a case without vasoformation and a new transcript variant.
      aug:
        au:
          Puls, Florian
          Niblett, Angela
          Clarke, Jade
          Kindblom, Lars-Gunnar
          McCulloch, Tom
        affil: Department of Musculoskeletal Pathology, Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham, UK, florian.puls@nhs.net.
      sug:
        subj:
          Carrier Proteins
          Proteins
          Hemangioma
          Hemangioma Pathology
          Phosphoproteins
          Adult
          Nucleotides
          Cystic Fibrosis Complications
          Immunohistochemistry
          In Situ Hybridization, Fluorescence
          Lung Transplantation
          Lymph Nodes Pathology
          Male
          Reverse Transcriptase Polymerase Chain Reaction
          Chromosome Disorders
          Adult: 19-44 years
          Male
      ab: Epithelioid hemangioendothelioma is a rare vascular tumor of borderline malignancy characterized by recurrent WWTR1-CAMTA1 gene fusions in approximately 90 % of cases. In addition, a recurrent YAP1-TFE3 gene fusion has been identified in WWTR1-CAMTA1 negative epithelioid hemangioendotheliomas. This subset has been reported as having a distinct morphology with more obvious vasoformation, voluminous eosinophilic cytoplasm, and TFE3 positivity on immunohistochemistry. We report a case of a YAP1-TFE3 translocated epithelioid hemangioendothelioma arising in a groin lymph node in a 29-year-old male. Plump spindle cell morphology and absence of vasoformation made correct diagnosis particularly difficult. Immunohistochemistry showed nuclear positivity for both ERG and TFE3, fluorescence in situ hybridization showed break apart for TFE3 and RT-PCR identified a YAP1 exon1 to TFE3 exon 6 transcript, a previously unreported fusion variant. Awareness of this solid morphology and variant fusion will aid in identification of future cases of this rare vascular tumor.
      pubtype: Academic Journal
      doctype:
        case study
        Journal Article
      ougenre: Article
    language: English
    refInfo:
    holdings:
      @attributes:
        islocal: N