| 摘要: |
| 硬纤维瘤(desmoid tumours,DT)是一种罕见的良性肿瘤,但可呈局部浸润性生长.FAP患者DT发病率显著高于一般人群,其发生、发展可能与手术、遗传学易感性和体内激素水平有关.50％的FAP相关性DT发生于腹腔内,常表现为无压痛、缓慢生长的肿块.临床表现包括腹痛、恶心、呕吐、腹泻等.肠系膜DT可导致肠梗阻、肠缺血、肾盂积水和瘘管.可活检或CT检查明确诊断.对于腹壁DT建议手术切除,而肠系膜DT的首选药物治疗,手术应慎重. |
| 关键词: 腺瘤性息肉病,结肠、硬纤维瘤、综述文献 |
| DOI：10.3724/SP.J.1008.2006.00541 |
|
| 基金项目: |
|
| Familial adenomatous polyposis-associated desmoid tumors |
| 楼征,于恩达,孟荣贵,LOU Zheng,YU En-da,MENG Rong-gui |
| () |
| Abstract: |
| Desmoid tumor （DT）, a rare benign tumor sometimes with local invasion, is more frequently seen in patients with familial adenomatous polyposis （FAP） than in the general public, which might be associated with surgical trauma, genetic predisposition and hormonal factors. Fifty percent of FAP-associated DTs are intra-abdominal, usually present as non-tender, slowly growing masses. The symptoms include abdominal pain, vomiting and diarrhea, etc. Mesenteric DT can cause obstruction and ischemia of intestine, hydronephrosis and fistula, Diagnosis can be made through biopsy or CT scanning. Surgery should be considered for the abdominal wall DTs and medicine is the first choice for mesenteric DTs, Surgery should be chosen with caution |
| Key words: adenomatous polyposis coli desmoid tumours review literature |
