【摘要】 韧带样瘤是一种深部筋膜或肌腱膜结构来源的低度恶性的纤维性肿瘤,发病率较低。腹壁外和腹壁韧带样瘤的治疗以外科手术为主。首选切缘2cm以上的广泛根治性切除,确保切缘阴性;无法达广泛切除或切缘阴性时,首选保全功能性手术。切缘阳性、复发性患者或无法手术者可考虑辅助放疗。腹腔内韧带样瘤多见于家族结肠腺瘤息肉病患者,手术切除复发率高,目前推荐首选大剂量的三苯氧胺和舒林酸治疗。由于韧带样瘤自然病程多变,药物治疗等疗效多不确切,包含观察等待的个体化治疗方案有望成为韧带样瘤的一种新的治疗模式。更多还原

【Abstract】 Desmoid tumor also known as aggressive fibromatosis,are rare fibroblastic tumors which are derived from deep fascia planes or muscuofascia structures.Resectable extra-abdominal and abdominal desmoid tumor should always be treated with the aim of achieving a negative margin through wide radical resection with margins above 2 cm.If this is not feasible,function-sparing surgical resection is suggested.Adjuvant radiation should be considered for patients with positive margins,recurrent tumors or unresectable diseases.Intra-abdominal desmoid tumor is common in patients with familial adenomatous polyposis.It has a high recurring rate after surgical resection and therefore a high dosage of tamoxifen and sulindac are recommended as first-line treatment.Due to the variable nature of the outcome and vague reaction to drug therapy,individualized treatments,including the wait and see policy,has been widely investigated and thought to be a promising strategy for the future.更多还原