Eastley N, McCulloch T, Esler C, Hennig I, Fairbairn J, Gronchi A, Ashford R
Leicester Royal Infirmary, The University of Leicester NHS Trust, Leicester, LE1 5WW, United Kingdom.
Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom.
Eur J Surg Oncol. 2016 Jul;42(7):1071-83. doi: 10.1016/j.ejso.2016.02.012. Epub 2016 Feb 19.
Extra abdominal desmoid fibromatosis is a complex condition with many recognised treatments including active observation, hormonal therapy, chemotherapy, radiotherapy and surgical resection. There is large variation in the natural history of individual desmoid tumours, with some cases progressing aggressively and others regressing spontaneously when observed alone. This combined with an absence of accurate clinical predictors of a desmoid tumour's behaviour has led to difficulties in identifying which patients would benefit most from aggressive treatment, and which could be adequately managed with a policy of active observation alone. This review explores the aetiology and common presentation of extra-abdominal desmoid fibromatosis including the condition's histopathological, clinical and radiological characteristics. The current evidence for potential predictors of desmoid tumour behaviour is also reviewed, along with the indications and evidence for the multitude of treatments available. We also summarise the published guidelines that are currently available for oncologists and surgeons managing extra-abdominal desmoid fibromatosis, and highlight some of the unanswered questions that need to be addressed to optimise the management of this condition.
腹外硬纤维瘤病是一种复杂的疾病，有多种公认的治疗方法，包括密切观察、激素治疗、化疗、放疗和手术切除。单个硬纤维瘤的自然病程差异很大，有些病例进展迅速，而有些病例单独观察时会自发消退。再加上缺乏硬纤维瘤行为的准确临床预测指标，导致难以确定哪些患者最能从积极治疗中获益，哪些患者仅通过密切观察策略就能得到充分管理。本综述探讨了腹外硬纤维瘤病的病因和常见表现，包括该疾病的组织病理学、临床和放射学特征。还综述了硬纤维瘤行为潜在预测指标的现有证据，以及多种可用治疗方法的适应证和证据。我们还总结了目前可供管理腹外硬纤维瘤病的肿瘤学家和外科医生使用的已发表指南，并强调了一些需要解决的未回答问题，以优化该疾病的管理。
