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6例色素性神经纤维瘤中男3例,女3例,年龄8～55岁.6例患者的皮损均表现为褐色斑片或斑块,3例皮损位于左颈部及躯干、四肢,1例皮损位于右腋窝及左上臂,1例皮损位于左前臂伸侧,1例皮损位于腰臀部,皮损2 cm×3 cm至30 cm×40 cm.3例合并Ⅰ型神经纤维瘤病.皮损组织病理主要表现为表皮轻度角化过度,基底层色素增加,真皮和皮下脂肪组织内可见肿瘤细胞呈弥漫性分布,肿瘤组织无包膜,主要由大量的梭形细胞构成.肿瘤组织中散在或簇集状分布黑素细胞,胞质内可见黑素颗粒.免疫组化显示S100(+)和波形蛋白(+).6例患者均未治疗.色素性神经纤维瘤少见,临床和组织病理上,需与Becker痣、先天性黑素细胞痣、色素性隆突性皮肤纤维肉瘤等鉴别.

Six patients with pigmented neurofibroma were reported.Of the 6 patients,3 were male and 3 were female,with the age varying from 8 to 55 years.All the patients presented with brown patches or plaques ranging from 2 cm × 3 cm to 30 cm × 40 cm in size.Lesions were located on the left lateral neck,trunk and extremities in 3 cases,on the right axillary fossa and left upper arm in 1 case,on the extensor aspect of the left forearm in 1 case,and on the waist and buttocks in 1 case.Three of these patients were complicated by neurofibromatosis type I.Histopathological examination revealed increased pigments in the basal layer,mild hyperkeratinization in the epidermis,and diffuse distribution of tumor cells in the dermis and subcutaneous adipose tissue.The tumor was non-capsulated,and consisted of plenty of spindle cells and a few scattered or clustered melanocytes with melanin granules in the cytoplasm.Immunohistochemical study showed positive staining for S100 and vimentin.None of the patients received treatment.Pigmented neurofibroma is rare in clinic,and should be clinically and histopathologically distinguished from Becker's nevus,congenital melanocytic nevus and pigmented dermatofibrosarcoma protuberans.