隆凸性皮肤纤维肉瘤

Two cases of congenital dermatofibrosarcoma protuberans

报告2例隆凸性皮肤纤维肉瘤。例1.女,8岁。右侧大腿结节6个月。皮肤科检查:右侧大腿内侧一红色结节,表面光滑,无溃疡,质韧,活动度一般,与周围组织分界不清,无触压痛。皮损组织病理:真皮内见大量致密分布的梭形细胞呈席纹样排列,局部向脂肪组织浸润,并见散在核分裂象。免疫病理:梭形肿瘤细胞CD34表达呈强阳性。诊断:儿童隆凸性皮肤纤维肉瘤。予局部扩大切除肿瘤,术后随访至今9个月未见复发或转移。例2.男,14岁。右侧胸腹部皮肤肿块14年。皮肤科检查:胸腹部右侧一红褐色浸润性肿块,表面光滑,质韧,与周围组织分界不清。皮损组织病理:表皮大致正常,真皮内大量梭形肿瘤细胞浸润,局部呈漩涡状排列,界限不清,并不规则地向皮下脂肪侵袭。免疫病理:梭形肿瘤细胞CD34表达呈强阳性。诊断:隆凸性皮肤纤维肉瘤。予Mohs显微外科手术切除肿瘤,局部皮瓣转移修复,术后随访至今35个月未见复发或转移。

Two cases of congenital dermatofibrosarcoma protuberans are reported. Case 1, an 8-year-old girl had had a nodule on the right thigh for 6 months. Physical examination showed a firm, immovable, asymptomatic red nodule on the right thigh. Biopsy of the nodule revealed spindle cells with mitotic figures arranged in storiform pattern in the dermis. Some spindle cells infiltrated into the subcutaneous adipose tissue. Tumor cells were strong positive for CD34. Childhood dennatofi- brosarcoma protuberans was the diagnosis. Extensive excision of tumor was performed. Neither recurrence nor metastasis was found during a follow-up period of 9 months. Case 2, a 14-year-old boy had had a mass on the right hypochondrium since birth. Dermatological examination revealed a red brown infiltrated mass, which affixed to the surrounding tissues, with smooth surface and firm texture, but unclear boundaries. Histopathological examination showed normal epidermis and infiltration of spindle-shaped tumor cells in the dermis. Spindle cells were arranged in a swirling pattern, and irregularly extended into the subcutaneous adipose tissue. Tumor cells were strong positive for CD34. A diagnosis of congenital dermatofibrosarcoma protu- berans was made. Mohs microsurgical excision was performed to remove the tumor. Skin defect was repaired with transplanta- tion of local skin flap. Neither recun＇ence nor metastasis was found during a follow-up period of 35 months after the opera- tion.