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以咽後腫塊表現之頸部脊索瘤-病例報告

Cervical Chordoma Presenting as a Retropharyngeal Mass - Case Report

摘要


脊索瘤是一罕見的惡性腫瘤,其具有緩慢生長、局部侵犯性及高復發性等特性。腫瘤起源於退化的脊索組織,臨床上好發於薦尾骨部,約佔50%,其次為斜坡,約佔35%,以及可動脊椎,約佔15%。男女發生比率約為2:1。可能發生在任何年齡,但是以中老年人居多。治療方法以完全切除為主,放射治療扮演輔助性的角色。本文報告一頸部脊索瘤之病例,檢查發現後咽壁有一隆起表面平滑之巨大腫塊。經電腦斷層掃描,發現腫瘤位於頸椎C1-C3之脊椎前部,而且向後侵蝕C2之脊椎體。經過近於完全的切除手術之後,仍有極少量腫瘤組織存留於C2脊椎體,術後給予放射治療。手術後於門診持續追蹤至今,雖仍有局部腫瘤殘存,但患者復原良好。

並列摘要


Chordomas are slow growing, locally invasive neoplasms which arise from remnants of the embryonic notochord. Chordomas are rare and generally located in the midline. We experience a case of cervical chordoma which is more rare and difficult to make different-tial diagnosis. A 75-year-old woman visited our hospital complaining of loud snoring and severe orthopnea for many months. Physical examination revealed a large, firm, immo-bile retropharyngeal mass, protruding anteriorly into the pharynx and covered by intact mucosa. Computed tomography revealed a well-demarcated soft tissue density and a septate mass located anterior to the spine which began at the level of nasopharynx and extended down to C3. The lesion had caused partial bony destruction of the vertebral body of C2. During operation frozen sections of the tumor showed typical histologic appearances of chordoma under microscope. Subtotal excision was performed which left few residual tumor at the right transverse foramen of C2. Adjuvant radiotherapy (50.4Gy) was administered after surgery. The patient was alive and free of symptoms despite the residual tumor in the vertebral body of C2 at 16 months of follow-up. Salvage surgery is recommended in patients with chordoma if any symptom recurs or the residual tumor grows. This case tells us cervical chordoma may be ignored by the patient for many months, since symptoms of dysphagia, hoarseness, or dyspnea may be slowly progress-sive. Chordoma should be included in the differential diagnosis in adult patients with a paraspinal, soft-tissue mass.

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