萎缩型隆突性皮肤纤维肉瘤误诊1例

Misdiagnosis of Atrophic Bulging Dermatofibrosarcoma:A Case Report and Literature Review

患者女,30岁,左侧季肋部暗红色结节6年,伴疼痛6个月余。患者曾以“硬皮病、结节病”治疗效果欠佳。查体见左侧季肋部可见不规则形状的暗红色结节,大小约为3.5 cm×1.9 cm,皮损表面部分萎缩,略有凹陷,边缘隆起,呈囊性、质软,皮损中央血管较为明显。皮损组织病理示:表皮变薄,真皮浅层炎症细胞浸润伴少许肿瘤细胞,真皮深部肿瘤细胞呈车轮状或席纹状,肿瘤细胞可见核分裂象,肿瘤细胞向脂肪组织浸润,分割脂肪细胞,呈现蜂窝状;免疫组织化学:CD34(+)。诊断:萎缩型隆突性皮肤纤维肉瘤。明确诊断后建议患者行扩大切除术及游离皮瓣转移术,患者拒绝并坚持保守治疗,现在随访中。

A 30-year-old female patient presented with dark red nodules on the left quarter of the ribs for six years,with pain for more than six months.She was treated for "scleroderma and sarcoidosis",with poor therapeutic effects.Physical examination showed dark red nodules with irregular shape on the left rib and with an area of about 3.5 cm×1.9 cm.The surface of the lesion was partially atrophied,slightly concave,and the edge was raised.It was cystic and soft,and in the center of the lesion the blood vessels were more obvious.Histopathology of skin lesions revealed thinning of the epidermis,infiltration of inflammatory cells in the superficial dermis with a small number of tumor cells,wheel-like or mat-like tumor cells in the deep dermis and mitotic figures,of tumor cells.Tumor cells infiltrated into adipose tissue,and divided fat cells,showing a honeycomb structure.Immunohistochemistry revealed CD34(+).Diagnosis of atrophic dermatoficrotic cutaneous fibrosarcoma was made.After a definitive diagnosis,patients were recommended to undergo extended resection and free flap transfer.The patient refused and insisted on conservative treatment,and is now being followed up.