A case of perivascular epithelioid cell tumor of the palatine mucosa

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  • KOMATSU Noriko
    Department of Oral and Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital
  • ABE Takahiro
    Department of Oral and Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital
  • SHIRAI Rena
    Department of Dentistry and Oral and Maxillofacial Surgery, International University of Health and Werfare Mita Hospital
  • TANIGUCHI Asako
    Department of Oral and Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital
  • ABE Masanobu
    Department of Oral and Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital
  • HOSHI Kazuto
    Department of Oral and Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital

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Other Title
  • 軟口蓋に発生した血管周囲類上皮細胞腫瘍の1例
  • ナンコウガイ ニ ハッセイ シタ ケッカン シュウイルイ ジョウヒ サイボウ シュヨウ ノ 1レイ

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Abstract

<p>Perivascular epithelioid cell tumor: PEComa is a group of tumors defined in the WHO classification of bone and soft tissue tumors as “mesenchymal tumors consisting of cells that are associated with blood vessel walls and express markers of melanocytes and smooth muscle”. We report a case of soft palate PEComa. The patient was a 17-year-old boy. The diagnosis of hemangiomatous fibrotic histiocytoma, which is considered to be a benign malignant intermediate tumor, was obtained at a previous doctor, and the patient was referred to our department because of the need for multidisciplinary treatment. A mass lesion with an ulcer was found in the midline of the soft palate. Contrast-enhanced MRI showed the findings of the separated nodule image in the soft palate. Preoperative evaluation of the previous specimen confirmed the diagnosis of PEComa. Since this case was a benign tumor with a relatively good prognosis, we performed resection with consideration for functional preservation. Although one year has passed since surgery, the patient has progressed well without recurrence. PEComa is extremely rare in the oral cavity. This is considered a valuable case for accumulating information in the future.</p>

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