Eastley N, Aujla R, Silk R, Richards C J, McCulloch T A, Esler C P, Ashford R U
Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester LE1 5WW, United Kingdom.
Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham NG5 1PB, United Kingdom.
Eur J Surg Oncol. 2014 Sep;40(9):1125-30. doi: 10.1016/j.ejso.2014.02.226. Epub 2014 Feb 22.
Desmoid fibromatosis (DF) carries a significant morbidity and a recognised mortality. Despite this there are currently limited diagnostic or treatment algorithms specific to cases of extra-abdominal DF. Historically surgical excision has formed the cornerstone of treatment. Recently however a paradigm shift has meant many practitioners now adopt a more conservative approach, placing emphasis on active surveillance, function preserving resections, and non-surgical oncologic therapies.
We performed an 8-year retrospective review of all cases of extra-abdominal DF managed within our region to assess the consistency of diagnostics, management and long-term outcome.
47 eligible cases were identified. Mean age at diagnosis was 41.3 years (1-81 years). Disease location and speciality of diagnosing practitioners were varied. Management was generally inconsistent. Variation was seen in imaging, biopsy techniques, MDT involvement and management. At a median follow up of 4.9 years our local recurrence rate was 19%.
The optimal management of DF is unknown. This has led to a lack of formalised guidance for practitioners managing this challenging condition, resulting in inconsistencies and areas for improvement in current management. We propose a diagnostic pathway which may improve consistency of care, reduce potentially unnecessary surgery and the associated morbidity, and significantly increase the rate of complete (R0) surgical resections when surgery is deemed appropriate whilst not significantly worsening oncological outcome. Specifically we propose all cases should be imaged appropriately (usually with MRI), undergo a planned biopsy (by radiologically guided core needle biopsy) and be managed centrally in conjunction with multidisciplinary sarcoma units.
韧带样纤维瘤病（DF）具有较高的发病率和公认的死亡率。尽管如此，目前针对腹外DF病例的诊断或治疗方案仍然有限。从历史上看，手术切除一直是治疗的基石。然而，最近的一种模式转变意味着许多从业者现在采取了更为保守的方法，强调积极监测、保留功能的切除术和非手术肿瘤治疗。
我们对本地区管理的所有腹外DF病例进行了为期8年的回顾性研究，以评估诊断、管理和长期结果的一致性。
共确定了47例符合条件的病例。诊断时的平均年龄为41.3岁（1 - 81岁）。疾病位置和诊断医生的专业各不相同。管理通常不一致。在影像学、活检技术、多学科团队参与和管理方面存在差异。在中位随访4.9年时，我们的局部复发率为19%。
DF的最佳管理方法尚不清楚。这导致了管理这种具有挑战性疾病的从业者缺乏正式的指导，从而导致当前管理中存在不一致和需要改进的地方。我们提出了一种诊断途径，这可能会提高护理的一致性，减少潜在的不必要手术及其相关发病率，并在认为手术合适时显著提高完全（R0）手术切除率，同时不会显著恶化肿瘤学结果。具体而言，我们建议所有病例都应进行适当的影像学检查（通常为MRI），进行计划好的活检（通过放射学引导的粗针活检），并与多学科肉瘤治疗团队联合进行集中管理。
