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Updated: Dec 31, 2025

Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
Ewing's sarcoma/peripheral primitive neuroectodermal tumor with extraskeletal myxoid chondrosarcoma-like areas: a
Zhe Wang1, Hongwei Dang2, Jinfang Jiang1
1Department of Pathology and Key Laboratory of Xinjiang Endemic and Ethnic Diseases (Ministry of Education), Shihezi University School of Medicine Shihezi 832002, Xinjiang, China.
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.

