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下顎蘭格罕氏細胞組織球增生症-病例報告

Langerhans Cell Histiocytosis of Mandible-A Case Report

摘要


蘭格罕氏細胞組織球增生症是一種罕見的低度惡性腫瘤,主要發生在孩童,局部骨頭疼痛為最常見的臨床症狀。本病例報告為一位48歲女性,主訴一個月前於診所拔牙,在最近一星期前右臉處開始有腫痛現象,因此前來門診尋求治療並接受切片手術,切片報告為右下顎蘭格罕氏細胞組織球增生症,之後病患於全身麻醉下施行右側舌骨上頸部淋巴廓清術、腫瘤廣泛性切除合併下顎骨截斷術,並同時使用游離腓骨皮瓣重建手術。術後恢復情況良好,出院後病患拒絕後續放射線治療、化學治療與新陳代謝科門診治療,目前仍在本科門診定期追蹤。

並列摘要


Langerhans cell histiocytosis is a rare low-grade malignancy that characterized by the infiltration and proliferation of Langerhans cells. LCH mainly occurs in children. However, its onset is rare in adults. Clinical symptoms vary, but local bone pain is the most frequent. We reported a case that a 48 year-old female patient complaining of severe pain of R't face presented with R't face swelling and tenderness for 1 week. About 1 month before she came to our clinic, she had tooth extracted at the local dental clinic. During the first interview, the patient reported that she had been diagnosed dietetic mellitus, central diabetic insipidus and amenorrhea one year ago but she did not have medical follow-up visits. The pathological report revealed Langerhan's cell histiocytosis of R't mandible. After systemic workup to determine the extent of the disease, she received neck dissection, tumor wide excision with mandibulectomy and free fibular flap reconstruction. During the hospitalization, we consulted the endocrinologist for central DI & DM control. She declined radiotherapy, chemotherapy and medication of DM & central DI postsurgery and had follow-up visit in our clinic only. One year following surgery, there were no signs of relapse.

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