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鼻咽橫紋肌肉瘤 — 病例報告

Nasopharyngeal Rhabdomyosarcoma - Case Report

摘要


橫紋肌肉瘤是兒童最常見的頭頸部軟組織惡性腫瘤。目前治療的觀念乃採合併手術、放射治療及多種藥物化學治療的多方式治療(multimodality therapy)為主。而手術中能將腫瘤完全切除的病人,則會有較好的治療效果。然而因為腫瘤所在的解剖位置及腫瘤本身侵犯的特性,欲進行腫瘤廣泛性的切除往往非常困難。我們經驗一鼻咽橫紋肌肉瘤合併側咽部侵犯的病例,經採下頜骨擺開法(mandibular swing),順利將腫瘤完全切除,臨床分期得以有效降低。術後病患並接受放射治療及化學治病,至今追蹤18個月,並無腫瘤復發的現象。由於術野良好,同時對於大血管能夠得到較好的控制,下頜骨擺開法提供了廣泛切除鼻咽腫瘤一個可靠的路徑。同時也因為它較少的破壞性和美觀上較好的考量,下頜骨擺開法更能符合手術治療發生於小兒中顏面部腫瘤的要求。合併下頜骨擺開法、放射治療及化學治療的多型式治療方式,提供了治療鼻咽橫紋肌肉瘤的一種良好選擇。

並列摘要


Rhabdomyosarcoma is the most common soft tissue malignancy of the head and neck in children. The management of this aggressive tumor has generally involved multimodal therapy that combines surgery, radiotherapy and chemotherapy. The most favorable treatment outcomes occur in patients in whom the neoplasm has been totally resected. Unfortunately, complete resection is often difficult because of the invasive nature of the tumor and its often anatomically concealed location. In this report, we describe and extensive nasopharyngeal rhabdomyosarcoma with parapharyngeal space involvement in which complete excision was achieved by using a mandibular swing technique. The stage of the tumor was significantly reduced after surgery. With postoperative radiotherapy and chemotherapy the patient has been free of recurrence over 18 months of follow-up. Given the excellent surgical field it provides and the improved control of the great vessels, the mandibular swing technique constitutes a reliable approach for wide field resection of nasopharygeal neoplasia. It is also less destructive and has fewer cosmetic complications for midfacial neoplasia in the young. Surgical resection using the mandibular swing technique in combination with radiotherapy and chemotherapy is an effective strategy for the management of nasopharyngeal rhabdomyosarcoma.

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