軟口蓋に発生した血管周囲類上皮細胞腫瘍の1例

• Published in: 日本口腔外科学会雑誌 Vol. 66; no. 8; pp. 371 - 375
• Main Authors: 安部, 貴大, 小松, 紀子, 阿部, 雅修, 谷口, 明紗子, 白井, 伶奈, 星, 和人
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.08.2020
• Subjects: 血管周囲類上皮細胞腫瘍
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.66.371

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.