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Sclerosing Rhabdomyosarcoma: Presentation of a Rare Sarcoma Mimicking Myoepithelial Carcinoma of the Parotid Gland and Review of the Literature

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Abstract

Sclerosing rhabdomyosarcoma (SRMS), a recently characterized variant of rhabdomyosarcoma, can pose a significant diagnostic challenge given its rarity and its histological similarity to other malignancies. SRMS is characterized by dense hyalinized or sclerosing collagenous matrix and a pseudovascular pattern of growth. SRMS shares histologic similarities with several mesenchymal tumors including: leiomyosarcoma, osteosarcoma, chondrosarcoma, angiosarcoma, and sclerosing epithelioid fibrosarcoma. We herein report a case of SRMS mimicking a myoepithelial carcinoma of the parotid gland. The tumor contained small, spindled, and epithelioid tumor cells lining pseudovascular spaces within a dense hyalinized stroma. Initial stains for keratins, S100 and p63 were negative. However the tumor cells showed desmin and myogenin positivity. The tumor was negative for FKHR gene rearrangements and showed no MDM2 gene amplification. This is the second case of SRMS to be diagnosed in the parotid gland highlighting the potential for misdiagnosis as a primary salivary gland epithelial malignancy.

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Authors and Affiliations

  1. Department of Diagnostic Sciences, School of Dental Medicine, University of Pittsburgh, Pittsburgh, PA, 15261, USA

    Blake M. Warner

  2. Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, 15261, USA

    Christopher C. Griffith & Raja R. Seethala

  3. Department of Pathology, York Laboratory Associates, York Hospital, York, PA, 17403, USA

    William D. Taylor

Authors
  1. Blake M. Warner
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  2. Christopher C. Griffith
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  3. William D. Taylor
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  4. Raja R. Seethala
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Correspondence to Blake M. Warner.

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Warner, B.M., Griffith, C.C., Taylor, W.D. et al. Sclerosing Rhabdomyosarcoma: Presentation of a Rare Sarcoma Mimicking Myoepithelial Carcinoma of the Parotid Gland and Review of the Literature. Head and Neck Pathol 9, 147–152 (2015). https://doi.org/10.1007/s12105-014-0540-x

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  • DOI: https://doi.org/10.1007/s12105-014-0540-x

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