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Adamantinoma-Like Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature

  • Diana Morlote
  • Shuko Harada
  • Brenessa Lindeman
  • Todd M. StevensEmail author
Original Paper

Abstract

Currently considered a variant of Ewing sarcoma, adamantinoma-like Ewing sarcoma is a rare malignancy that shows classic Ewing sarcoma-associated gene fusions but also epithelial differentiation. Here we present the 6th reported case of adamantinoma-like Ewing sarcoma involving the thyroid gland. Sections of the thyroid tumor from a 20-year old woman showed sheets, lobules and trabeculae of primitive, uniform, small round blue cells that diffusely expressed pankeratin, p40 and CD99. Fluorescent in situ hybridization revealed an EWSR1 gene rearrangement and an EWSR1-FLI1 fusion was detected by RT-PCR. Neck lymph nodes were not involved, and the patient was treated with a Ewing sarcoma chemotherapy protocol and radiation and is disease free 7 months after surgery. The unusual histology and immunohistochemical profile of adamantinoma-like Ewing sarcoma makes diagnosis and classification very challenging. We also present a literature review of adamantinoma-like Ewing sarcoma involving the thyroid.

Keywords

Adamantinoma-like Ewing sarcoma Thyroid Sarcoma Ewing sarcoma Molecular diagnostics 

Notes

Compliance with Ethical Standards

Conflict of interest

The authors have no conflicts of interest or financial relationships to disclose.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Ethical Approval

This article does not contain any studies with animals performed by any of the authors.

Informed Consent

Informed consent was waived for this study as part of the corresponding author’s IRB at UAB (X170428002).

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of PathologyUniversity of Alabama at BirminghamBirminghamUSA
  2. 2.Department of SurgeryUniversity of Alabama at BirminghamBirminghamUSA

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