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Adamantinoma-like Ewing Sarcoma of the Nasal Cavity: A Rare Clinical Presentation with Deceptive Histopathologic Features

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Abstract

Ewing sarcoma (ES) is a malignant round cell tumour (MRCT) that usually involves bone and soft tissue of young and paediatric populations. ES of the head and neck region is uncommon. Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of ES that shows complex epithelial differentiation on histopathology and immunohistochemistry (IHC). It demonstrates a t(11;22) translocation and EWSR1- FLI1 fusion. Most documented cases of ALES of the head and neck region were initially misdiagnosed as epithelial tumours. We present a rare case of ALES of the nasal cavity in a young female. The patient subsequently underwent chemotherapy and showed an excellent response. Awareness of this entity is important for pathologists and oncologists due to its distinct therapeutic and prognostic implications. We propose performing upfront NKX2.2 and CD99 IHC studies, as well as other lineage specific IHC markers, in any poorly differentiated MRCT of head and neck region.

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Acknowledgements

The authors would like to acknowledge Ms Sangeeta Arora, and Mr Arvind Bhuker for their technical assistance in performing IHC stains.

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The authors have no relevant financial or non-financial interests to disclose.

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Authors and Affiliations

  1. Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, Delhi, 110085, India

    Sunil Pasricha, Saloni Pahwa, Ankur Kumar, Gurudutt Gupta, Anila Sharma, Garima Durga, Meenakshi Kamboj & Anurag Mehta

  2. Department of Radiology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India

    Ankush Jajodia

  3. Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, Delhi, 110085, India

    Sumit Goyal

  4. Department of Surgical (Head and Neck) Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, Delhi, 110085, India

    Mudit Agarwal

Authors
  1. Sunil Pasricha
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  2. Saloni Pahwa
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  3. Ankush Jajodia
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  4. Sumit Goyal
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  6. Ankur Kumar
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  7. Gurudutt Gupta
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  8. Anila Sharma
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  9. Garima Durga
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  10. Meenakshi Kamboj
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  11. Anurag Mehta
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Corresponding author

Correspondence to Sunil Pasricha.

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Pasricha, S., Pahwa, S., Jajodia, A. et al. Adamantinoma-like Ewing Sarcoma of the Nasal Cavity: A Rare Clinical Presentation with Deceptive Histopathologic Features. Head and Neck Pathol 16, 865–869 (2022). https://doi.org/10.1007/s12105-021-01396-4

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  • DOI: https://doi.org/10.1007/s12105-021-01396-4

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