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Clinical Challenge
November 2016

New Facial Weakness After 5 Years of Facial Asymmetry

Author Affiliations
  • 1Department of Pathology and Laboratory Medicine, NorthShore University HealthSystem, Evanston, Illinois
  • 2Section of Otolaryngology–Head and Neck Surgery, Department of Surgery, University of Chicago, Chicago, Illinois
  • 3Division of Otolaryngology, NorthShore University HealthSystem, Evanston, Illinois
JAMA Otolaryngol Head Neck Surg. 2016;142(11):1121-1122. doi:10.1001/jamaoto.2015.3289

A man in his 30s presented with a steady 5-year progression of facial asymmetry and new-onset left-sided facial weakness, pain, and otalgia. Physical examination revealed no palpable parotid mass and no overlying skin changes. Neurologic examination showed incomplete left eye closure with a grade 5 of 6 on the House-Brackmann scale. Treatment with steroids and antiviral agents marginally improved facial strength and allowed eye closure. Magnetic resonance imaging showed subcutaneous nodules focally infiltrating the superficial parotid gland with evidence of perineural spread along the labyrinthine segment of CN VII. Fine-needle aspiration (FNA) with ultrasonographic guidance was nondiagnostic. An open excisional biopsy demonstrated dense subcutaneous tissue overlying and invading the parotid gland. Intraoperative frozen section revealed a spindle cell tumor. A superficial parotidectomy with facial nerve dissection and preservation was performed. Histopathologic examination revealed a subcutaneous mass with stellate extension into and around the parotid gland. The tumor consisted of bland fusiform tumor cells in a storiform pattern near inconspicuous hyalinized vessels (Figure, A and B). Immunohistochemical staining demonstrated diffuse positivity for CD34 (Figure, C), positivity for bcl-2, and negativity for CD99 and S-100. Fluorescent in situ hybridization (FISH) further characterized the tumor with a translocation t(17;22)(q21;q13) and corresponding COL1A1/PDGFB fusion protein (Figure, D).

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