惡性周邊神經髓鞘瘤

我們報告一位62歲男性，於就醫前六個月發現一個漸漸增大的背部腫塊。他本身並無其他病史，也無腫瘤或癌症家族史。磁振造影掃描發現在其上背部有一約7x5x3立方公分的軟組織腫瘤，並沿著左側第四神經孔侵犯到脊柱內。開刀後，病理切片證實為惡性周邊神經髓鞘腫瘤，且標本的腫瘤邊緣是乾淨的。開刀後他接受局部輔助性放射治療（6000cGy/30次）。兩年後，胸部X光發現有肺臟轉移，且背部開刀處也出現腫瘤復發。於是他再次接受了背部局部腫瘤切除及肺臟轉移腫瘤切除手術。之後他接受四次全身性化學治療。術後五個月做磁振造影檢查追蹤，發現背部仍有局部腫瘤復發。目前已持續在門診追蹤八個月。惡性周邊神經髓鞘瘤是一種惡性紡錘細胞肉瘤，和神經纖維瘤、第一型多發性神經纖維瘤症和以前接受過放射治療的部位有密切相關。此類病患最重要的預後因子是腫瘤大小，是否可完整取出腫瘤，及是否合併第一型多發性神經纖維瘤症。對於出現在深部軟組織的腫瘤，尤其是長在神經附近又快速變大時，我們應小心此腫瘤之可能性，並根據病患之狀況給予適當治療，提高病患之存活率。

關鍵字

惡性周邊神經髓鞘瘤；磁振造影掃描；肉瘤

並列摘要

We reported a 62-year-old man who developed a progressively enlarged firm mass in the upper back over 6 months. The patient had no family history or stigmata of neurofibromatosis type 1. Magnetic resonance images showed a 7 cm deep soft tissue tumor in the upper back with extension to left neural foramen of 4th thoracic spine. After surgical resection, the pathological examination confirmed a malignant peripheral nerve sheath tumor with free tumor margin. Local radiotherapy (6000cGy/30 times) was given after the surgery. However, lung metastasis and local tumor recurrence were found after two years. Therefore, he received another surgery for local and metastatic tumor resection, and postoperative adjuvant chemotherapy. Local tumor recurrence was still noted after 5 months. He kept regular follow-up at out-patient clinics for 8 months. Malignant peripheral nerve sheath tumor is an aggressive spindle cell sarcoma that is closely associated with neurofibromas, neurofibromatosis type 1 and from the sites previously irradiated. The tumor size, the presence of neurofibromatosis, and total resection are the most important prognostic indicators. A large deep soft tissue tumor with rapid enlargement and perineural spread should prompt the consideration of this tumor, which helps early diagnosis and adequate treatment to improve the survival of these patients.

並列關鍵字

Malignant peripheral nerve sheath tumor ； Magnetic resonance imaging ； Sarcoma

國際替代計量

惡性周邊神經髓鞘瘤

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