Ewing's sarcoma/peripheral primitive neuroectodermal tumor with extraskeletal myxoid chondrosarcoma-like areas: a case report

Int J Clin Exp Pathol. 2019 Oct 1;12(10):3940-3943. eCollection 2019.

Abstract

Introduction: Ewing's sarcoma (EWS)/peripheral primitive neuroectodermal tumor (pPNET) (EWS/pPNET) is a group of highly aggressive small round cell tumors of the bone or soft tissue with high metastatic potential and an aggressive course in children and young adults. EWS/pPNET microscopically does not often have a myxoid background.

Case description: We report an EWS/pPNET, which exhibited an unusual morphology with cells having an acidophilic cytoplasm set in a myxoid background, raising the possibility of extraskeletal myxoid chondrosarcoma (EMC). A reverse transcription-polymerase chain reaction analysis confirmed the presence of an EWS-FLI1 fusion transcript.

Conclusions: Morphology, immunohistochemistry, and molecular assays may be necessary to avoid a potential diagnostic pitfall as EWS/pPNET with a myxoid background may histologically resemble an EMC.

Keywords: Ewing’s sarcoma/peripheral primitive neuroectodermal tumor; extraskeletal myxoid chondrosarcoma; immunohistochemical; molecular assays; morphology; small round blue cells.

Publication types

  • Case Reports