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Osteosarcoma arising from fibrous dysplasia of the mandible- case report

起源於下顎骨纖繩性發育不良之骨肉瘤-病例報告

摘要


惡性轉變很少發生於骨之纖維性發育不良,但惡性轉變可能發生於單骨性或多骨性纖維性發育不良。我們報告一例發生於一位非症候群相關,且未經過放射線治療之34歲男性患者之下顎骨纖維性發育不良,惡性轉變成骨肉瘤之病例。患者於6年前曾跌倒過,經頭部及胸部之放射線檢查,發現於左額骨、左下顎骨和左助骨有多骨性纖維性發育不良。因為病變沒有快速生長跡象,患者當時並未針對多骨性纖維性發育不良治療。患者於6年後,於下顎骨纖維性發育不良處發生惡性轉變,腫瘤快速生長成一巨大腫塊,該腫瘤連同左頰、左頦皮膚和軟組織、左下顎骨和左舌一起以外科手術切除。切除後之缺陷,由整形外科醫師以一人工髁和一游離腓骨皮辦修補切除之左下顎骨,和一前側面股辦修補切除之左頰、左頦組織。切下之標本經組織病理檢查後,發現為一高惡性度之造骨細胞骨肉瘤。患者手術後復原良好,經6個月後,腫瘤無復發之現象。

並列摘要


Malignant transformation is a rare complication of fibrous dysplasia (FD) of the bone. It may occur in patients with either monostotic or polyostotic FD. We present a case of malignant transformation of polyostotic FD into a mandibular osteosarcoma in a non-syndrome-associated 34-year-old male patient in the absence of radiation therapy. The patient was diagnosed as having polyostotic FD involving the left frontal bone, the left mandible, and the left ribs during a radiographic examination of the head and chest in an episode of falling down 6 years ago. Since there was no active growth of the lesions, he received no treatment for the polyostotic FD at that time. Malignant change in the fibrous dysplastic left mandible occurred 6 years after initial diagnosis of the disease, resulting in a huge tumor with surface ulceration of the left mandible. The tumor together with the left cheek and chin skin and soft tissue, the left mandible, and the left tongue was excised. The surgical defect was reconstructed using an artificial condyle and a free fibular osteocutaneous flap for the excised left mandible, and with an anterolateral thigh flap for the excised left cheek and chin tissue by plastic surgeons. The histopathologic examination of the specimen revealed a high-grade osteoblastic osteosarcoma. The postoperative course was smooth, and no postoperative recurrence was found 6 months after the initial surgery.

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