Multicentric Desmoid Tumors: A Case Report and Literature Review

多發家族性纖維瘤病：一病例報告及文獻回顧

黃培銘(Pei-Ming Huang) ； 張逸良(Yih-Leong Chang) ； 李元麒(Yung-Chie Lee)

《胸腔醫學》 17卷1期 (2002/03) Pp. 78-83

摘要

侵犯性纖維瘤病是一種良性、罕見、及時常緩慢生長的腫瘤，對於化學及放射線療法常有抵抗性。他們常發生在任何肌肉筋膜層，且有向鄰接的組織侵潤，變大的趨勢，和引起機能的限制或者疼痛，但不轉移。
我們報告一個四十八歲的女性病例，在22年來她因為纖維瘤病接受了十二次的手術。之前她有腹壁的纖維瘤病，並且這個遺傳性的纖維瘤疾病影響到三代的親屬，但是並無家族性大腸息肉症的大腸息肉的特色。除此之外，此腹壁的纖維瘤病成腹內的纖維瘤病，最後一次的手術則於1997年10月因為長在腹部外靠在右側肩胛骨的纖維瘤病而開刀。我們對於腫瘤區域進行廣泛的切除，截至目前為止並無腫瘤再復發的情形發生。這種多發性且多次復發的纖維瘤病，由腹壁小腸發生，最後胸壁也發生是相當罕見的。本文針對纖維瘤病的臨床、組織學，及處理方法上加以討論說明。

關鍵字

纖維瘤病 ； 多發性 ； 家族性大腸息肉症

並列摘要

Aggressive fibromatosis (desmoid tumor) is a pathologic benign, uncommon, and often slowly growing fibrous tumor that is highly resistant to therapy. This type of tumor may arise in any musculoaponeurotic structure, and has a tendency to infiltrate adjacent tissues, become large in size, and cause functional limitation and/or pain, but does not metastasize.
We report a 48-year-old woman who underwent operation twelve times for recurrent and multicentric desmoid tumors, and briefly review the literature. The patient had an abdominal wall tumor 22 years ago, and has a hereditary desmoid disease which has affected a three generations in her family, but lacks the colonic features of familial adenomatous polyposis (FAP). In addition, a desmoid tumor at the mesentery involving the small intestine was also noted. The last operation, involving a wide excision of a right scapula tumor in the posterior area of the right chest wall, was performed in 1997. There is no local recurrence at present. Multiple episodes of recurrence (12 times), and multicentric occurrence, including an intra-abdominal presentation of the tumor, focal infiltration of the small bowel, and involvement of the chest wall, were unusual features for desmoid tumors. The clinical and histological features of desmoid tumors are presented here, with a discussion on management options.

並列關鍵字

desmoid tumor ； multicentric ； familial adenomatous polyposis