隆突性皮肤纤维肉瘤伴纤维肉瘤转化1例。

Q4 Medicine
Ceskoslovenska patologie Pub Date : 2020-01-01
Jan Hrudka, Martin Charvát, Petr Grossmann, Zdeněk Kinkor
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引用次数: 0

摘要

隆突性皮肤纤维肉瘤是一种非常罕见的真皮和皮下局部侵袭性肿瘤,表现出特有的形态和染色体易位(17;22)(q21;q13)与COL1A1-PDGFB基因融合。肿瘤几乎从不转移,完全切除预后良好。大约在10%的病例中,皮肤纤维肉瘤经历纤维肉瘤转化并伴有转移性疾病和较差的预后。在本文中,我们提出了一个病例的男性患者皮下肿瘤在背部区域,其中小活检导致梭形细胞肉瘤的诊断。然而,只有在根治性手术材料中对整个肿瘤进行组织病理学检查,才发现有纤维肉瘤转化的隆突性皮肤纤维肉瘤。肿瘤的两个组成部分都表现出特征性的遗传改变。在DFSP中纤维肉瘤成分的识别对预后很重要。区分发生在隆突性皮肤纤维肉瘤内的纤维肉瘤和新生的纤维肉瘤具有重要的治疗意义:转移性或不能手术的DFSP伴纤维肉瘤转化的患者可能受益于伊马替尼治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: a case report.

Dermatofibrosarcoma protuberans is a quite rare local aggressive tumor of dermis and subcutis, revealing characteristic morphology and chromosomal translocation (17; 22)(q21;q13) with gene fusion COL1A1-PDGFB. The tumour almost never metastasizes and complete excision signs an excellent prognosis. Approximately in 10% of cases, dermatofibrosarcoma undergoes a fibrosarcomatous transformation associated with metastatic disease and worse prognosis. In this paper, we refer a case of a male patient with subcutaneous tumor in back region, in which the small biopsy lead to diagnosis of a spindle cell sarcoma. However, only the histopathological examination of the entire tumor in the material from the radical surgery detected the dermatofibrosarcoma protuberans with fibrosarcomatous transformation. Both components of the tumor showed the characteristic genetic alteration. Identification of fibrosarcomatous component within the DFSP matters in prognosis. Distinction between fibrosarcoma arising within the dermatofibrosarcoma protuberans and fibrosarcoma arising de novo is of therapeutic consequence: the patients with metastatic or inoperable DFSP with fibrosarcomatous transformation may profit form imatinib treatment.

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来源期刊
Ceskoslovenska patologie
Ceskoslovenska patologie Medicine-Medicine (all)
CiteScore
0.40
自引率
0.00%
发文量
17
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