Multicentric Malignant Peripheral Nerve Sheath Tumors of Small Intestine and Thigh: Report of a Case

小腸原發性周邊神經鞘惡性腫瘤(MPNST)係一種極爲罕見之疾病，本文報告一例在左大腿後側及小腸（空腸）同時發生周邊神經鞘惡性腫瘤，這是一名59歲男性病患，在七個月內分別因爲左大腿後側皮下腫塊及威脅生命的血便先後兩次到醫院就診，並接受外科手術治療。第二次住院接受剖腹探查手術，進行空腸腫瘤切除手術及端對端空腸空腸吻合術。關於組織學病理診斷，發生於左大腿後側之惡性周邊神經鞘腫瘤爲「典型周邊神經鞘惡性腫瘤」，發生於小腸（空腸）之惡性周邊神經鞘腫瘤爲「類上皮周邊神經鞘惡性腫瘤」。病人於空腸手術後一個月發生多重器官遠處轉移，三個月後死亡。這是台灣首例小腸原發性周邊神經鞘惡性腫瘤。

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並列摘要

Primary malignant peripheral nerve sheath tumor (MPNST) of the small bowel is an extremely rare disease. We report a multicentric MPNSTs of the left thigh and jejunum in a 59-year-old male, who visited our hospital twice in seven months because of a subcutaneous lump on the posterior aspect of the left thigh and life-threatening melena respectively. Laparotomy was performed for the small bowel tumor and a segment of the jejunum containing the tumor near its midportion was resected with end-to-end jejunojejunostomy. Histologically, the tumor which had been excised seven months previously was diagnosed as classical MPNST occurring in the left thigh, and that resected this time, as epithelioid MPNST occurring in the jejunum. Distant metastasis to multiple organs occurred one month after resection of the jejunal MPNST, and the patient died three months later. This is the first case of an MPNST arising in the small intestine reported in Taiwan.

並列關鍵字

malignant peripheral nerve sheath tumor ； multicentric tumors ； small bowel malignancy

國際替代計量

Multicentric Malignant Peripheral Nerve Sheath Tumors of Small Intestine and Thigh: Report of a Case

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