Lips D J, Barker N, Clevers H, Hennipman A
Department of Surgery, Jeroen Bosch Hospital, Den Bosch, The Netherlands.
Eur J Surg Oncol. 2009 Jan;35(1):3-10. doi: 10.1016/j.ejso.2008.07.003. Epub 2008 Aug 21.
Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize. The choice of treatment remains surgical resection with or without radiotherapy, and is characterized by high recurrence rates. Better understanding of the aetiology of aggressive fibromatosis is needed to be able to develop new treatment strategies to cope with the high recurrence rates.
Relevant studies were identified through a search of the electronic databases PubMed/ Medline. The following search terms were used: 'aggressive fibromatosis', 'desmoid tumor', 'adenomatous polyposis coli', 'APC', 'beta-catenin', 'Wnt', 'Wingless' and 'Wnt/Wingless'. Studies were selected for review on the basis of abstract reading. A hand search was performed by checking reference lists in selected articles.
The neoplastic nature of aggressive fibromatosis and the role of the adenomatous polyposis coli (APC) and beta-catenin signaling cascade in driving the onset and progression of this disease are discussed.
Mutations in either the APC or beta-catenin genes are likely to be a major driving force in the formation of these desmoid tumors. More research is needed to develop new treatment strategies.
侵袭性纤维瘤病（同义词：韧带样瘤）是一种偶发性的成纤维细胞肿瘤性增殖，起源于肌筋膜平面，形成侵袭性生长的肿块且无转移能力。治疗选择仍然是手术切除，可联合或不联合放疗，其特点是复发率高。需要更好地了解侵袭性纤维瘤病的病因，以便能够制定新的治疗策略来应对高复发率。
通过检索电子数据库PubMed/Medline来识别相关研究。使用了以下检索词：“侵袭性纤维瘤病”、“韧带样瘤”、“腺瘤性息肉病 coli”、“APC”、“β-连环蛋白”、“Wnt”、“无翅型”和“Wnt/无翅型”。根据摘要阅读来选择研究进行综述。通过检查所选文章的参考文献列表进行手工检索。
讨论了侵袭性纤维瘤病的肿瘤性质以及腺瘤性息肉病 coli（APC）和β-连环蛋白信号级联在驱动该疾病发生和发展中的作用。
APC 或β-连环蛋白基因的突变可能是这些韧带样瘤形成的主要驱动力。需要更多研究来制定新的治疗策略。
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