PDF(Pubmed)
Abstract：
Desmoid tumors (DTs) are deep fibroblastic neoplasms that arise from musculo-aponeurotic stromal elements. DTs may result in significant morbidity by infiltrating vital anatomic structures. Their mortality is often due to the local aggressiveness, most commonly when intra-abdominal in location. Some indolent DTs can be observed expectantly; infiltrative tumors require an aggressive and multidisciplinary approach and are offered conservative therapies such as nonsteroidal anti-inflammatory drugs or antiestrogens when surgery is not feasible. Comparably, chemotherapy is considered for those cases not amenable to surgery or radiation. Bowel resection and at times intestinal transplantation may be necessary. However, DTs may recur postsurgery making long-term management of these patients. Herein, we review the genetics, clinical presentations, outcomes, and treatments of DTs.
摘要：
纤维瘤（DTs）是由肌肉-腱膜基质元素引起的深部成纤维细胞肿瘤。DTs可能通过浸润重要的解剖结构而导致严重的发病率。他们的死亡往往是由于当地的侵略性，最常见的是腹内位置。可以预期观察到一些惰性DTs；浸润性肿瘤需要积极和多学科的方法，并且在手术不可行时提供保守治疗，例如非甾体抗炎药或抗雌激素。比较而言,对于那些不适合手术或放疗的病例，考虑化疗。肠切除和有时肠移植可能是必要的。然而,DTs可能会在手术后复发，从而对这些患者进行长期管理。在这里，我们回顾遗传学，临床表现,结果，和DTs的处理。
