Díaz-Cascajo C, Borrego L, Bastida-Iñarrea J, Borghi S
Pathology Department, Hospital Nuestra Señora del Pino, Las Palmas de Gran Canaria, Spain.
Am J Dermatopathol. 1996 Aug;18(4):403-8. doi: 10.1097/00000372-199608000-00013.
Giant cell fibroblastoma is a rare mesenchymal neoplasm of unknown origin and uncertain clinical course. The neoplasm has been considered by some authors as a juvenile variant of dermatofibrosarcoma protuberans. We report a patient with giant cell fibroblastoma, now 28 months following surgical removal, in which the neoplasm was characterized histologically by a proliferation of spindle-shaped cells intermixed with pseudovascular channels called "angiectoid spaces." The spaces were lined by large cells with pleomorphic nuclei intermixed with multinucleated cells. Immunohistochemically, the tumor stained diffusely for vimentin and CD34, a surface glycoprotein expressed by some mesenchymal neoplasms including dermatofibrosarcoma protuberans. We postulate a mechanism of formation of the angiectoid spaces based on histopathological findings in serially sectioned portions of the neoplasm. Positive staining of tumor cells for CD34 supports a possible relationship of the neoplasms with dermatofibrosarcoma protuberans.
巨细胞成纤维细胞瘤是一种起源不明、临床病程不确定的罕见间叶性肿瘤。一些作者认为该肿瘤是隆突性皮肤纤维肉瘤的青少年型变体。我们报告一例巨细胞成纤维细胞瘤患者,手术切除后已过去28个月,该肿瘤在组织学上的特征是梭形细胞增生,并伴有称为“血管样间隙”的假血管通道。这些间隙内衬有核多形性的大细胞,并混有多核细胞。免疫组织化学显示,肿瘤弥漫性表达波形蛋白和CD34,CD34是一种表面糖蛋白,一些间叶性肿瘤包括隆突性皮肤纤维肉瘤可表达该蛋白。我们根据肿瘤连续切片部分的组织病理学发现推测了血管样间隙的形成机制。肿瘤细胞CD34染色阳性支持该肿瘤与隆突性皮肤纤维肉瘤可能存在关联。