An intra-abdominal small round cell neoplasm with features of primitive neuroectodermal and desmoplastic round cell tumor and a EWS/FLI-1 fusion transcript

Hum Pathol. 1997 Apr;28(4):502-9. doi: 10.1016/s0046-8177(97)90042-3.

Abstract

We report an intra-abdominal round cell tumor in a young man which exhibited the light and electron microscopic appearance of a peripheral primitive neuroectodermal tumor (PNET), in addition to the clinical and topographic characteristics, desmoplasia and a complex immunophenotypic profile of the intra-abdominal desmoplastic round cell tumor (DSRCT). Reverse transcription polymerase chain reaction revealed a EWS/FLI-1 fusion transcript as in PNET/Ewing's sarcoma, instead of the EWS/WT1 transcript of DSRCT. The tumor was also strongly positive for the mic2 protein. This is a unique case of a hybrid tumor arising in the peritoneal cavity of a young male. The existence of such a hybrid tumor in this location suggests that DSRCT and PNET may be related and possibly share a common histogenesis.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Neoplasms / pathology*
  • Abdominal Neoplasms / ultrastructure
  • Adult
  • Biomarkers / analysis
  • Blotting, Western
  • Carcinoma, Small Cell / pathology*
  • Carcinoma, Small Cell / ultrastructure
  • DNA-Binding Proteins / metabolism
  • Desmin / analysis
  • Fatal Outcome
  • Humans
  • Immunohistochemistry
  • Keratins / analysis
  • Male
  • Microscopy, Electron
  • Neuroectodermal Tumors, Primitive / pathology*
  • Neuroectodermal Tumors, Primitive / ultrastructure
  • Oncogene Proteins, Fusion / metabolism*
  • Polymerase Chain Reaction
  • Proto-Oncogene Protein c-fli-1
  • Proto-Oncogene Proteins*
  • Sarcoma, Ewing / metabolism*
  • Tomography, X-Ray Computed
  • Trans-Activators / metabolism

Substances

  • Biomarkers
  • DNA-Binding Proteins
  • Desmin
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Protein c-fli-1
  • Proto-Oncogene Proteins
  • Trans-Activators
  • Keratins