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四肢和躯干黏液纤维肉瘤:多学科方法可实现良好的局部控制率。

Myxofibrosarcoma of the extremity and trunk: a multidisciplinary approach leads to good local rates of LOCAL control.

作者信息

Ghazala C G, Agni N R, Ragbir M, Dildey P, Lee D, Rankin K S, Beckingsale T B, Gerrand C H

机构信息

Newcastle Upon Tyne Hospitals NHS Foundation Trust, Freeman Hospital, Newcastle Upon Tyne NE7 7DN, UK.

Newcastle University, Newcastle Upon Tyne NE1 7RU, UK.

出版信息

Bone Joint J. 2016 Dec;98-B(12):1682-1688. doi: 10.1302/0301-620X.98B12.37568.

Abstract

AIMS

Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours.

PATIENTS AND METHODS

Patients with a primary or recurrent MFS who were treated surgically in our unit between 1997 and 2012 were included in the study. Clinical records and imaging were reviewed. A total of 50 patients with a median age of 68.4 years (interquartile range 61.6 to 81.8) were included. There were 35 men; 49 underwent surgery in our unit.

RESULTS

The lower limb was the most common site (32/50, 64%). The mean size of the tumours was 8.95 cm (1.5 to 27.0); 26 (52%) were French Fédération Nationale des Centres de Lutte Contre le Cancer grade III. A total of 21 (43%) had positive margins after the initial excision; 11 underwent further excision. Histology showed microscopic spread of up to 29 mm beyond macroscopic tumour. Local recurrence occurred in seven patients (14%) at a mean of 21 months (3 to 33) and 15 (30%) developed metastases at a mean of 17 months (3 to 30) post-operatively.

CONCLUSION

High rates of positive margins and the need for further excision makes this tumour particularly suited to management by multidisciplinary surgical teams. Microscopic tumour can be present up to 29 mm from the macroscopic tumour in fascially-based tumours. Cite this article: Bone Joint J 2016;98-B:1682-8.

摘要

目的

黏液纤维肉瘤(MFS)是一种恶性软组织肉瘤,其特征表现为四肢出现无痛性缓慢生长的肿块。局部浸润性生长意味着局部复发风险较高。我们回顾了自身经验,以对这些肿瘤的切除策略及多学科团队在其管理中的作用提出建议。

患者与方法

纳入1997年至2012年间在本单位接受手术治疗的原发性或复发性MFS患者。回顾临床记录和影像学资料。共纳入50例患者,中位年龄68.4岁(四分位间距61.6至81.8)。男性35例;49例在本单位接受了手术。

结果

下肢是最常见的部位(32/50,64%)。肿瘤平均大小为8.95 cm(1.5至27.0);26例(52%)为法国国立癌症中心联合会III级。初次切除后共有21例(43%)切缘阳性;11例接受了进一步切除。组织学检查显示显微镜下肿瘤扩散范围超出肉眼可见肿瘤达29 mm。7例患者(14%)出现局部复发,平均时间为21个月(3至33个月),15例(30%)在术后平均17个月(3至30个月)发生转移。

结论

高切缘阳性率及进一步切除的必要性使得这种肿瘤特别适合由多学科手术团队进行管理。在基于筋膜的肿瘤中,显微镜下肿瘤可存在于距肉眼可见肿瘤达29 mm处。引用本文:《骨与关节杂志》2016年;98 - B:1682 - 1688。

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