Perivascular Epithelioid Cell Tumor Involving the Mandible-A Case Report

下顎血管周圍上皮樣細胞瘤－病例報告

何宗訓(Tsung-Hsun Ho) ； 李正喆(Jang-Jaer Lee)

《臺灣口腔顎面外科學會雜誌》 32卷3期 (2021/09) Pp. 197-206

摘要

Perivascular epithelioid cell tumors (PEComas), is a family of mesenchymal tumors occurring in any part of the human body. PEComas consist of perivascular epithelioid cells with a clear/granular cytoplasm and central round/oval nucleus with small nucleoli. Expression of Melanocytic markers and myogenic markers in immunohistochemistry is the hallmark of the disease. We report a case of a benign perivascular epithelioid cell tumor presenting as a slow-growing asymptomatic mandibular lesion in a 14-year-old Asian adolescent. The initial biopsy result shows small-blue-round-cell tumor. We performed a composite resection of the tumor due to extensive intraosseous invasion of tumor. Based on pathological analysis, the definitive diagnosis of the tumor is a PEComa. Occurrence of PEComas in the head and neck region is uncommon and intraosseous involvement pertaining to its clinical behavior is rare. To our knowledge, this is the first benign PEComas involving the mandible. Recurrence has not been reported seven years after surgery.

關鍵字

Perivascular epithelioid cell tumor ； PEComas ； Mesenchymal tumors ； Mandibular neoplasm

並列摘要

血管周圍上皮樣細胞瘤是屬於間葉細胞瘤的一種，會發生在全身任何的地方。血管周圍上皮樣細胞瘤主要由血管周圍的上皮樣細胞所組成，其細胞有透明或顆粒狀的細胞質及中正且小的圓型或卵圓型細胞核。在免疫染色中，呈現黑色素性及肌原性的生物標記是此類腫瘤的重要特徵。本篇案例報告記錄一位十四歲亞洲青少年患有無症狀且生長緩慢的良性血管周圍上皮樣細胞瘤。初步的切片檢查診斷為小藍圓細胞腫瘤。因為腫瘤延伸廣泛且侵犯大範圍的下顎骨，因此施行廣泛性腫瘤切除合併下顎骨部份切除手術。術後病理診斷為良性血管周圍上皮樣細胞瘤。此類腫瘤罕見於頭頸部，且為骨内腫瘤的比例更為稀少。以目前的文獻回顧，本病例為第一例被發表之下顎骨骨内良性血管周圍上皮樣細胞瘤。患者術後追蹤七年，並無復發之跡象。

並列關鍵字

血管周圍上皮樣細胞瘤 ； 間葉細胞瘤 ； 下顎腫瘤