Mediastinum Chordoma: A Case Report

縱隔腔脊索瘤－病例報告

張蒼育(Choon-Yuk Chong) ； 蘇建銘(Jang-Ming Su) ； 楊國坤(Kok-Khun Yong) ； 吳子卿(Tzu-Chin Wu)

《胸腔醫學》 24卷1期 (2009/02) Pp. 54-59

https://doi.org/10.29806/TM.200902.0008

摘要

脊索瘤是一種緩慢生長，起源於殘餘胚性背索的腫瘤，但是對局部組織破壞性很強，可發生於顱骨底(35%)，頸，胸，腰脊椎(2.3-15%)，和薦椎(50%)。胸廓的脊索瘤同樣罕見。在此一病例報告中，我們報告一個21歲女性病患，有一個緩慢生長達14年的左側縱隔腔腫瘤。其X光表現像良性的腫瘤，即邊緣清楚，鈣化及生長緩慢，而延遲病患的及時診斷和治療。
她兩年前開始出現左手乾燥的症狀。最近出現胸後背部痛。超音波指引抽吸術病理檢查報告為小葉和圓狀的大腫瘤細胞於軟骨樣基質。特殊免疫染色呈現對S-100蛋白，細胞角蛋白(cytokeratin)，波形蛋白(vimentin)陽性。這些檢查證實為惡性脊索瘤，病患隨後接受此縱隔腔腫瘤的完全切除，同時進行C7，T1-T3的局部切骨術及填充骨泥。病患術後接受50Gy局部立體放射線治療，至今5個月也沒有局部復發。

關鍵字

縱隔腔脊索瘤

並列摘要

Chordoma is a slow-growing yet locally aggressive malignant neoplasm of the bone derived from remnants of the embryonic notochord. Thoracic chordoma is even rarer. It may occur at the skull base (35%), at the cervical, thoracic and lumbar spine (2.3-15%), and at the sacral regions (50%). We present a 21 year-old girl who had a slow-growing left-side mediastinal tumor for 14 years. The chest X-ray film showed a benign-looking, small-sized, well-circumscribed tumor with calcification; the benign appearance on the chest X-ray delayed her prompt diagnosis and treatment.
The patient began to experience left hand dryness 2 years previously, and a recent onset of back pain. Echo-guided aspiration revealed cords and lobules of large tumor cells in the chondroid stroma. Immunohistochemical staining was positive for S-100 protein, cytokeratin and vimentin. These observations indicated a malignant chordoma. She received a total surgical resection of the tumor, together with a partial osteotomy of C7 and T1-T3. The area of bone substance loss was filled with cement. Adjuvant stereotatic radiotherapy with 50 Gy was given. No local recurrence was seen after 5 months.

並列關鍵字

mediastinum chordoma