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Solitary Extramedullary Plasmacytoma of the Anterior Mediastinum-A Case Report

前縱膈腔的單獨骨髓外漿細胞瘤-病例報告

摘要


單獨骨髓外漿細胞瘤源自血液的漿細胞,屬於非何杰金氏淋巴瘤的一類。單獨骨髓外漿細胞瘤佔所有漿細胞腫瘤的4%,可能發生在身體的任何部位,主要發生於上消化呼吸道,發生於縱膈腔及胸壁者相當罕見。此腫瘤對放射線治療具高度敏感性,一系列的文獻報告均指出放射線治療局部控制率可達80-100%,少於30%的病例會進展成多發性骨髓瘤,10年存活率約在70%,其預後比單獨性骨頭漿細胞瘤或多發性骨髓瘤好。我們報告一名主訴前胸壁有腫塊的病人,胸部電腦斷層檢查發現一巨大均質的前縱膈腔腫瘤往前延伸到前胸壁但沒有合併骨頭的破壞。對胸壁腫瘤施行細針抽吸及超音波導引穿刺術的組織病理報告為骨髓外漿細胞瘤。血清蛋白質電泳顯示免疫球蛋白A輕鏈的高度表現。經檢查後並沒有多發性骨髓瘤的證據。最後的診斷是分泌免疫球蛋白A輕鏈的前縱膈腔的單獨骨髓外漿細胞瘤。

並列摘要


The solitary extramedullary plasmacytoma (SEP) is derived from plasma cells and is included in the category of non-Hodgkin’s lymphoma. SEPs make up 4% of all plasma cell tumors and can occur in any site in the body, but are found mainly in the upper aerodigestive tract (UAD). Chest wall and mediastinal SEPs are extremely rare. Local control rates of 80-100% with radiotherapy are consistently reported due to the highly radiosensitive character of SEP. Less than 30% of SEPs progresses to multiple myeloma (MM), and the 10-year survival rate is around 70%. SEP has a better prognosis than solitary bone plasmacytoma (SBP) and MM. We report a patient with a mediastinal mass extending to the anterior chest wall without bony destruction. Fine-needle aspiration and echo-guided biopsy of the chest wall mass revealed a plasmacytoma. Serum protein electrophoresis demonstrated a spike in immunoglobulin A (IgA) lambda chains. Investigations for multiple myeloma were negative. The final diagnosis was SEP of the anterior mediastinum extending to the anterior chest wall.

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