An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma

Luigi Insabato, Elia Guadagno, Valentina Natella, Anna Somma, Michel Bihl, Antonio Pizzolorusso, Pier P aolo Mainenti, Gaetano Apice, Luigi Tornillo

Research output: Contribution to journalArticle

Abstract

Very recently a new designation of "Malignant Neuroectodermal Gastrointestinal Tumor" has been proposed for an aggressive form of neuroectodermal tumor with features similar to that of Clear Cell Sarcoma of Soft Tissue, however without a melanocytic differentiation. Also known as "clear cell sarcoma-like tumors of the gastrointestinal tract", these tumors show some features strongly suggesting an origin from a gastrointestinal neuroectodermal precursor cell unable to differentiate along the melanocytic lineage. They occur mainly in young and middle-aged adults, and have a poor prognosis with a high rate of liver and lymphnode metastases. Histologically they are composed of epithelioid or oval-to spindle cells with a sheet-like or nested pattern of growth, strongly positive for neural markers (S-100, SOX10, and vimentin) and negative for the melanocytic ones. EWSR1 gene rearrangements including EWSR1-ATF1 or EWSR1-CREB1 GENE fusions are typically assessed in these tumors. Here we report a case of malignant neuroectodermal gastrointestinal tumor which immunophenotypically unusually expressed FLI-1, occurring in a 29-year-old man with a previous medical history of Ewing sarcoma. We finally suggest that this case might be a further evidence of a link between these two entities.

Original languageEnglish
Pages (from-to)688-692
Number of pages5
JournalPathology Research and Practice
Volume211
Issue number9
DOIs
Publication statusPublished - Sep 1 2015

Keywords

  • Clear cell sarcoma-like gastric tumor

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Insabato, L., Guadagno, E., Natella, V., Somma, A., Bihl, M., Pizzolorusso, A., Mainenti, P. P. A., Apice, G., & Tornillo, L. (2015). An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma. Pathology Research and Practice, 211(9), 688-692. https://doi.org/10.1016/j.prp.2015.06.001