Choudhury Hasan, Abidoye Oluseyi
Internal Medicine, Northeast Georgia Medical Center Gainesville, Gainesville, USA.
Cureus. 2023 Mar 3;15(3):e35723. doi: 10.7759/cureus.35723. eCollection 2023 Mar.
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.
硬纤维瘤是起源于结缔组织的局部侵袭性良性肿瘤，被归类为不发生转移的软组织肉瘤。其名称源于希腊语单词desmos，意为肌腱样。这些肿瘤也被称为侵袭性纤维瘤病，其自然病史不可预测，会因风险因素而异。由于其局部侵袭性和高局部复发率，它们被当作肉瘤来治疗。硬纤维瘤发生的原因尚不明确，其临床病程也不可预测。疾病进展也因多种综合征风险因素而有很大差异。目前，对于这种肿瘤类型的最佳治疗方法尚无科学共识。治疗可能是观察、全身治疗、手术或放射治疗的组合。在此，我们描述了一例散发型硬纤维瘤女性患者，该患者对他莫昔芬和舒林酸治疗成功产生反应。
