PDF(Pubmed)
Abstract：
Desmoid tumors are locally aggressive benign tumors arising from connective tissue and are classified as soft tissue sarcomas that do not metastasize. The name is derived from the Greek word desmos that means tendon-like. These tumors are also known as aggressive fibromatosis and have an unpredictable natural history that varies depending on risk factors. They are treated as sarcomas because of their locally aggressive nature and a high local recurrence rate. The causes behind desmoid tumor development are enigmatic and their clinical course is unpredictable. Disease progression also varies widely depending on multiple syndromic risk factors. At this time, there is no scientific consensus over best treatment practices for this tumor type. Treatment can potentially be a combination of observation, systemic therapy, surgery or radiation therapy. Here, we have described a case of a female patient with a sporadic desmoid tumor that successfully responded to tamoxifen and sulindac.
摘要：
纤维瘤是由结缔组织引起的局部侵袭性良性肿瘤，被分类为不转移的软组织肉瘤。该名称源自希腊单词desmos，意为类似肌腱。这些肿瘤也被称为侵袭性纤维瘤病，具有不可预测的自然史，取决于风险因素。由于其局部侵袭性和高局部复发率，因此将其视为肉瘤。纤维瘤发展背后的原因是神秘的，其临床过程是不可预测的。疾病进展也根据多种综合征危险因素而变化很大。此时,对于这类肿瘤的最佳治疗实践,目前尚无科学共识.治疗可能是观察的结合，全身治疗,手术或放射治疗。这里,我们已经描述了一例女性患者，该患者患有散发性硬纤维瘤，对他莫昔芬和舒林酸成功应答。
