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伴有转移的肢端隆突性皮肤纤维肉瘤

Acral dermatofibrosarcoma protuberans with metastases.

作者信息

Skoll P J, Hudson D A, Taylor D A

机构信息

Department of Anatomical Pathology, Groote Schuur Hospital, and the University of Cape Town, South Africa.

出版信息

Ann Plast Surg. 1999 Feb;42(2):217-20.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor that occurs rarely on the extremities. This tumor has a particular propensity for local recurrence following seemingly adequate excision. Metastases are rare, and usually follow repeated local recurrences. On rare occasions, fibrosarcomatous change may arise in a DFSP, and appears to affect adversely the prognosis. The adequacy of the initial resection is the most important prognostic factor; however, suggested margins vary between 1.5 and 5 cm. The ideal margin of resection on the foot is unknown. We report a patient with recurrent, acral DFSP with fibrosarcomatous change and pulmonary parenchymal metastases in a 48-year-old black male.

摘要

隆突性皮肤纤维肉瘤(DFSP)是一种罕见的皮肤肿瘤,极少发生于四肢。该肿瘤在看似充分切除后有特别高的局部复发倾向。转移罕见,通常发生在多次局部复发之后。在极少数情况下,DFSP中可能会出现纤维肉瘤样改变,这似乎会对预后产生不利影响。初始切除的充分性是最重要的预后因素;然而,建议的切缘在1.5至5厘米之间。足部理想的切除切缘尚不清楚。我们报告了一名48岁黑人男性患者,患有复发性、肢端DFSP,伴有纤维肉瘤样改变和肺实质转移。

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