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原发性或复发性腹外部位的硬纤维瘤病:仅观察评估的治疗。

Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only.

机构信息

Orthopedic Surgery and Traumatology Department, Cochin Hospital, AP-HP, René-Descartes University, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.

出版信息

Orthop Traumatol Surg Res. 2010 Dec;96(8):884-9. doi: 10.1016/j.otsr.2010.07.007. Epub 2010 Nov 13.

DOI:10.1016/j.otsr.2010.07.007
PMID:21075698
Abstract

INTRODUCTION

Extra-abdominal desmoid fibromatosis (EADF) is a benign tumoral condition, classically managed by more or less radical and sometimes mutilating excision. This treatment strategy is associated with a recurrence rate of nearly 50% according to various reports.

HYPOTHESIS

EADF may show spontaneous stabilization over time.

METHODS

A retrospective series of 26 cases of EADF managed by simple observation was studied to assess spontaneous favorable evolution and identify possible factors impacting evolution. Eleven cases were of primary EADF with no treatment or surgery, and 15 of recurrence after surgery with no adjuvant treatment. MRI was the reference examination during follow-up.

RESULTS

Twenty-four cases showed stabilization at a median 14 months; there were no cases of renewed evolution after stabilization. One primary tumor showed spontaneous regression, and one recurrence still showed evolution at end of follow-up (23 months). The sole factor impacting potential for evolution was prior surgery. No radiologic or pathologic criteria of evolution emerged from analysis.

DISCUSSION

The present series, one of the largest dedicated to EADF managed by observation, confirmed recent literature findings: a conservative "wait-and-see" attitude is reasonable and should be considered when large-scale resection would entail significant functional or esthetic impairment.

LEVEL OF EVIDENCE

Level IV, retrospective study.

摘要

引言

腹外型硬纤维瘤病(EADF)是一种良性肿瘤性疾病,经典的治疗方法是或多或少地进行激进且有时会导致残缺的切除。根据不同的报道,这种治疗策略与近 50%的复发率相关。

假设

EADF 可能会随着时间的推移而自发稳定。

方法

我们研究了 26 例仅通过观察进行管理的 EADF 病例的回顾性系列,以评估自发的有利演变并确定可能影响演变的因素。11 例为原发性 EADF,无治疗或手术史,15 例为手术后复发且无辅助治疗。在随访期间,MRI 是参考检查。

结果

24 例在中位数为 14 个月时表现出稳定;在稳定后没有出现重新演变的情况。1 例原发性肿瘤出现自发消退,1 例复发病例在随访结束时(23 个月)仍在演变。唯一影响潜在演变的因素是先前的手术。分析未得出任何影像学或病理学的演变标准。

讨论

本系列研究是最大规模的专注于观察管理的 EADF 之一,证实了最近的文献发现:当大范围切除会导致显著的功能或美观损害时,保守的“观望”态度是合理的,应该被考虑。

证据水平

IV 级,回顾性研究。

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