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Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

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Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.

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Authors and Affiliations

  1. Department of Pathology, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Pathology 401, Baltimore, MD, 21287, USA

    Morgan L. Cowan & Justin A. Bishop

  2. Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Pathology 401, Baltimore, MD, 21287, USA

    Justin A. Bishop

  3. Department of Pathology, Southern California Permanente Medical Group, Woodland Hills, CA, USA

    Lester D. Thompson

  4. Department of Anatomic Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA

    Marino E. Leon

Authors
  1. Morgan L. Cowan
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  2. Lester D. Thompson
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  3. Marino E. Leon
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  4. Justin A. Bishop
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Correspondence to Morgan L. Cowan.

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Cowan, M.L., Thompson, L.D., Leon, M.E. et al. Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature. Head and Neck Pathol 10, 161–166 (2016). https://doi.org/10.1007/s12105-015-0647-8

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  • DOI: https://doi.org/10.1007/s12105-015-0647-8

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