摘要
侵袭性纤维瘤(Aggressive fibromatosis,AF)又称硬性纤维瘤,局部侵袭性生长,但无远处转移能力的软组织肿瘤,手术切除是主要治疗方法,复发率高。发病机制不明确,早期检测也一直无法解决。文献报道突变的β-catenin基因腺瘤肠息肉基因(APC)可以促进侵袭性纤维瘤发生,AD—AM12、FAP、WISP1和SOX11基因在侵袭性纤维瘤中选择性高表达。
Aggressive fibromatosis, also known as desmoid tumor, is a locally invasive soft tissue lesion arising from connective tissues. There is no metastasis to distant sites. The primary treatment remains surgical resection. It is characterized by high recurrence rates. The underlying mechanisms of development and regulation are unknown. Early detection is elusive. Recent studies have showed that mutations in either APC or β-catenin gene are probably a major driving force. And ADAM12, FAP, WISP1 and SOX11 are selectively over-expressed.
出处
《中华小儿外科杂志》
CSCD
2016年第2期156-160,共5页
Chinese Journal of Pediatric Surgery
关键词
纤维瘤病
侵袭型
基因
Fibromatosis, aggressive
Genes