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目的:探讨萎缩性隆突性皮肤纤维肉瘤(DFSP)的临床及组织病理特征。方法:对西京皮肤医院确诊的5例萎缩性DFSP进行临床及组织病理学分析。结果:萎缩性DFSP主要表现为缓慢生长的界限清楚的萎缩性斑块,部分皮损可伴有皮下结节或外生性结节。临床上萎缩性DFSP容易误诊,其中4例误诊为硬斑病。萎缩性DFSP组织病理表现为真皮明显变薄,真皮内肿瘤细胞不形成典型的席纹状排列,而是平行于表皮,肿瘤细胞可以不浸润脂肪组织。免疫组化显示肿瘤细胞CD34阳性,CD68、S-100蛋白阴性。结论:相对于经典型DFSP而言,萎缩型DFSP的皮损不呈经典的结节性改变,而是表现为萎缩斑,容易误诊。组织病理表现为真皮萎缩,肿瘤细胞不呈席纹状排列,容易误诊为瘢痕或其他纤维细胞肿瘤。
Objective: To investigate the clinical and histopathological features of atrophic cutaneous fibrosarcoma (DFSP). Methods: Clinical and histopathological analysis of 5 atrophic DFSP diagnosed in Xijing Skin Hospital was performed. RESULTS: Atrophic DFSP mainly manifested as clear-cut atrophic plaques with slow growth. Some lesions may be associated with subcutaneous nodules or extrinsic nodules. Clinically atrophic DFSP misdiagnosed, of which 4 cases were misdiagnosed as sclerotiorum. Atrophic DFSP histopathology showed significantly thinning of the dermis, dermal tumor cells do not form a typical arrangement of stripes, but parallel to the epidermis, tumor cells can not infiltration of adipose tissue. Immunohistochemistry showed that the tumor cells CD34 positive, CD68, S-100 protein negative. CONCLUSION: Compared with the classic DFSP, the lesions of atrophic DFSP do not show the classic nodular changes, but appear as atrophy spots, which are easily misdiagnosed. Histopathology showed dermal atrophy, tumor cells were not arranged in rows of stripes, easily misdiagnosed as scar or other fibrocystic tumors.