Primary Pulmonary Myxoid Sarcoma: A Newly Described Entity - Report of a Case and Review of the Literature

Academic Article


  • © The Author(s) 2017. Primary pulmonary myxoid sarcoma (PPMS) is a recently described, exceedingly rare low-grade lung sarcoma that tends to present in young females as an endobronchial mass and shows evidence of an EWSR1-CREB1 fusion. Herein, we present a case of PPMS with fluorescence in situ hybridization (FISH) analysis for EWSR1 and CREB1 rearrangements. An 80-year-old woman presented with an endobronchial, multinodular tumor exhibiting spindle, ovoid and epithelioid cells arranged in reticular/lattice-like and alveolar-like patterns in a myxoid background. The tumor showed focal epithelial membrane antigen immunoreactivity as well as an Alcian blue-positive stroma that was sensitive to digestion with hyaluronidase. EWSR1 and CREB1 rearrangements were detected by break-apart FISH probes. The patient showed persistence of disease 36 months after diagnosis and was discharged to hospice care. We contribute with a report of an additional case of this very unusual entity and perform a brief review of the literature published so far on the subject.
  • Digital Object Identifier (doi)

    Author List

  • Prieto-Granada CN; Ganim RB; Zhang L; Antonescu C; Mueller J
  • Start Page

  • 518
  • End Page

  • 525
  • Volume

  • 25
  • Issue

  • 6