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Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature

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Abstract

In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas. We report the case of a 39-year-old Caucasian male with a large heterogeneously enhancing mass in the pancreatic body. Pathologic examination showed a malignant round blue cell tumor diffusely positive for CD99, chromogranin, and synaptophysin; Ki-67 proliferation index was greater than 80%. FISH showed EWSR1 gene rearrangement in 90% of cells and Archer FusionPlexTM-targeted RNA sequencing analysis identified the EWSR1-FLI1 fusion transcript. The diagnosis of EFT of the pancreas was rendered. Unfortunately, the patient had minimal improvement and was transitioned to oral pain medications to continue care at a different institution.

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Acknowledgements

The authors would like to thank Dr. Meera Hameed from the Department of Pathology, Memorial Sloan Kettering Cancer Center, NY, for her diagnostic expertise.

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Authors and Affiliations

  1. Department of Pathology, Northwell Health, 6 Ohio Drive, suite 202, Lake Success, NY, 11042, USA

    Miglena K. Komforti & Rebecca M. Thomas

  2. Department of Radiology, Northwell Health, Manhasset, NY, USA

    Evgeniya Sokolovskaya & Catherine A. D’Agostino

  3. Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Ryma Benayed

Authors
  1. Miglena K. Komforti
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  2. Evgeniya Sokolovskaya
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  3. Catherine A. D’Agostino
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  4. Ryma Benayed
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  5. Rebecca M. Thomas
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Corresponding author

Correspondence to Miglena K. Komforti.

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The authors declare that they have no conflict of interest.

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Komforti, M.K., Sokolovskaya, E., D’Agostino, C.A. et al. Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature. Virchows Arch 473, 361–369 (2018). https://doi.org/10.1007/s00428-018-2344-y

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  • DOI: https://doi.org/10.1007/s00428-018-2344-y

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