title: An intra-abdominal small round cell neoplasm with features of primitive neuroectodermal and desmoplastic round cell tumor and a EWS/FLI-1 fusion transcript.
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.
authors.affiliation: Department of Anatomic Pathology, National Naval Medical Center, Bethesda, MD, USA.
authors.forename: R L
authors.initials: RL
authors.lastname: Katz
authors.forename: M
authors.initials: M
authors.lastname: Quezado
authors.forename: A M
authors.initials: AM
authors.lastname: Senderowicz
authors.forename: L
authors.initials: L
authors.lastname: Villalba
authors.forename: W B
authors.initials: WB
authors.lastname: Laskin
authors.forename: M
authors.initials: M
authors.lastname: Tsokos
mesh_terms: D000008:Abdominal Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D000328:Adult; D015415:Biomarkers / Q000032:analysis; D015153:Blotting, Western; D018288:Carcinoma, Small Cell / Q000473:pathology* / Q000648:ultrastructure; D004268:DNA-Binding Proteins / Q000378:metabolism; D003893:Desmin / Q000032:analysis; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D007633:Keratins / Q000032:analysis; D008297:Male; D008854:Microscopy, Electron; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology* / Q000648:ultrastructure; D015514:Oncogene Proteins, Fusion / Q000378:metabolism*; D016133:Polymerase Chain Reaction; D051789:Proto-Oncogene Protein c-fli-1; D011518:Proto-Oncogene Proteins; D012512:Sarcoma, Ewing / Q000378:metabolism*; D014057:Tomography, X-Ray Computed; D015534:Trans-Activators / Q000378:metabolism
chemical_list: D015415:Biomarkers; D004268:DNA-Binding Proteins; D003893:Desmin; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D011518:Proto-Oncogene Proteins; D015534:Trans-Activators; D007633:Keratins