色素型隆突性皮肤纤维肉瘤8例光镜、免疫组织化学及电镜观察

目的 探讨色素型隆突性皮肤纤维肉瘤(DFSP)临床病理特征和鉴别诊断。方法 对8例色素型DFSP进行了光镜和免疫组化染色观察，其中4例做电镜观察。结果 色素型DFSP患者的年龄、性别、肿瘤部位和肿瘤生长方式与普通型DFSP相同。随访结果3例发生复发，无1例发生转移。4例肿瘤切面见浅黑色或黑色斑。所有病例既可见典型的DFSP组织形态学特征，同时又见散在性分布、数量多少不等的黑色素细胞。本组6例瘤组织显示普通型DFSP图像，2例部分区域显示纤维肉瘤型DFSP图像，偶可见血管壁平滑肌增生形成的肌样结节和黏液样变性区。Fontana染色黑色素细胞呈阳性，Perls染色则为阴性。梭形瘤细胞呈vim和CD34阳性，CD34阳性具有一定辅助诊断价值，S-100蛋白和NSE阴性；黑色素细胞呈S-100蛋白和Vim阳性；纤维肉瘤型DFSP区Ki—67阳性表达率高于普通型DFSP区。电镜观察：可见梭形纤维母细胞样细胞和含有许多成熟黑色素小体黑色素细胞。结论 色素型DFSP是一种少见的DFSP亚型，临床病理特征与普通型DFSP对比有许多共同之处，需与动脉瘤样型纤维组织细胞瘤、弥漫性神经纤维瘤、细胞性蓝痣和促结缔组织增生性黑色素瘤鉴别。

Pigmented dermatofibrosarcoma protuberans: light microscopic, immunohistochemical and ultrastructural observation of 8 cases

Purpose To investigate clinicopathological characteristics and differential diagnosis of pigmented dermatofibrosarcoma protuberans (P DFSP). Methods Eight cases of P DFSP were observed by histologic and immunohistochemical staining, in which four cases was examined by electron microscopy. Results The age, sex, location, clinical manifestation and course were similar to conventional DFSP. Follow up showed three patients with recurrence, but no patient developed metastases. Microscopically, the cut surface in four tumors were pale black or black specks due to deposited melanin. Grossly, all tumors displayed typical pictures of spindle shaped neoplastic cells arranged in storiform pattern. A varying number of scattered melanocytes were also presented within these tumors. Six tumors were classified as conventional type, and other two as fibrosarcomatous type, one of them myoid nodules was detected as well. Immunohistochemically, spindle shape neoplastic cells were positive for CD34 and vimentin, but negative for S 100 protein and NSE. Melanocytes expressed also S 100 protein and vimentin. The percentage of Ki 67 positive cells was higher in P DFSP with fibrosarcomatous area than that without. Ultrastructurally, spindle fibroblastic like cells and melanocytes containing numerous mature melanosomes were identified. Conclusions P DFSP is a rare subtype of DFSP. Both P DFSP and conventional DFSP share many common clinical and pathological features. It should be distinguished from aneurysmal fibrous histiocytoma, diffuse neurofibroma, cellular blue nevus and desmoplastic melanoma.