A Unique Case of Malignant Fibrous Histiocytoma of the Small Intestine with Metastasis of the Bilateral Adrenal Glands and Small Intestine Intussusception

在此報告一位因小腸惡纖維組織細胞瘤造成兩側腎上腺轉移及小腸套疊阻塞之病例。病患為60歲女性，臨床上因為胃腸不適及體重減輕而到本院求診。腹部電腦斷層掃描顯示兩側腎上腺腫塊、上消化道鋇劑檢查顯示有小腸內填充缺陷及左側腎上腺腫塊造成之外部壓迫。在為病患施行腎上腺腫塊之電腦斷層導引穿刺切片檢查時，順利取得檢體標本。但是同時發現左上腹部之小腸形狀和前次腹部電腦斷層掃描有顯著差異，出現管腔內脂肪密度之區域，因而判斷有小腸套疊之情形，並建義開刀治療。手術結果發現兩側腎上腺腫塊及小腸15公分套疊，套疊前端有小腸腫塊作為小腸套疊之導引點。病理報告顯為小腸惡性纖維組織細胞瘤造成兩側腎上腺轉移及小腸套疊。因為腫瘤所造成小腸套疊，是比較罕見小腸阻塞原因。小腸套疊常在電腦斷層下表現特異的表徵，即管腔內脂肪密度之區域，代表套疊部分之腸繫膜脂肪。在影像檢查中，確認此種具有相當特異性表徵有助於影像診斷及決定治療方針。

關鍵字

惡性纖維組織細胞瘤；套疊；腎上腺轉移；阻塞

並列摘要

We report a unique case of malignant fibrous fibrous histiocytoma of the small intestine with metastasis of the bilateral adrenal glands and intussusception of the small intestine caused by the primary tumor. Malignant fibrous histiocytoma is a common soft tissue neoplasm, but only one case of primary and one case of metastatic malignant fibrous histiocytoma of the gastrointestinal tract producing intussu-sception has ever been reported from the articles available. Our patient was a 60-year-old female who was admitted with the complaints of gastrointestinal upset and body weight loss. Abdominal CT showed bilateral adrenal masses and follow-through small bowel series revealed a filling defect in the jejunum. Repeated CT on CT-guided biopsy revealed mesenteric fat within the lumen of jejunum, which was characteristic of intussusception. Surgery was performed and pathological analysis confirmed primary malignant fibrous histiocytoma with metastasis of the bilateral adrenal glands and intussusception induced by the primary tumor as leading point. We reviewed past articles reporting intussusception in adults and other similar cases available to increase our understanding.

並列關鍵字

malignant fibrous histiocytoma ； intussusception ； adrenal metastases ； obstruction

國際替代計量

A Unique Case of Malignant Fibrous Histiocytoma of the Small Intestine with Metastasis of the Bilateral Adrenal Glands and Small Intestine Intussusception

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