A case of malignant peripheral nerve sheath tumor of the mandibular gingiva

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  • MAKISHI Shoko
    Department of Oral and Maxillofacial Functional Rehabilitation, Graduate School of the Ryukyus
  • MATAYOSHI Akira
    Department of Oral and Maxillofacial Surgery, University of the Ryukyus Hospital
  • MIYAMOTO Sho
    Department of Oral and Maxillofacial Surgery, University of the Ryukyus Hospital
  • HIRANO Husahiro
    Department of Oral and Maxillofacial Surgery, University of the Ryukyus Hospital
  • NAKASONE Toshiyuki
    Department of Oral and Maxillofacial Surgery, University of the Ryukyus Hospital
  • NAKAMURA Hiroyuki
    Department of Oral and Maxillofacial Functional Rehabilitation, Graduate School of the Ryukyus Department of Oral and Maxillofacial Surgery, University of the Ryukyus Hospital

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  • 下顎歯肉に発生した悪性末梢神経鞘腫瘍の1例
  • カガク シニク ニ ハッセイ シタ アクセイ マッショウ シンケイ サヤ シュヨウ ノ 1レイ

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Abstract

<p>Malignant peripheral nerve sheath tumors (MPNSTs) are very rare malignant neoplasia that arise from the peripheral nervous system and show nerve sheath differentiation. We report a case of primary malignant peripheral nerve sheath tumor of the mandibular gingiva.</p><p>　A 53-year-old male was referred to our hospital presenting with a mass and pain in the mandibular gingiva. He had no appreciable disease and paralysis of the lower lip.</p><p>　Computed tomography showed resorption of the mandible but no obvious lymph node metastasis.</p><p>　Biopsy revealed a possible invasive unusual neuroectodermal tumor. Surgical resection was performed and the frozen section showed negative margins.</p><p>　Histology revealed atypical spindle cells with clear chromatin and clear nucleolus which had proliferated and loosely infiltrated, but the cell boundaries were unclear. Immunohistochemical staining showed positive results for S-100 protein, p53, and vimentin, but negative results for pan-cytokeratin (AE1 / AE3), Melan A, and desmin. The Ki-67 labeling index was 10%. Accordingly, we diagnosed a low-grade peripheral nerve sheath tumor of the mandibular gingiva.</p><p>　There were no signs of recurrence or metastasis at two years postoperatively. MPNSTs have poor prognoses and require careful follow-up.</p>

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