Mao Rong-Jun, Li Qi-Ming, Guo Yue-Ming, Li Wei-Qiang, Fan Chang-Shu, Zhu Xiong-Zeng
Department of Pathology, Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan 528000, China.
Zhonghua Bing Li Xue Za Zhi. 2010 Nov;39(11):752-6.
to study the clinicopathological features, imaging characteristics, immunophenotypes and differential diagnosis of giant cell angioblastoma (GCAB).
a case of GCAB in the left middle-upper tibia and fibula was studied by light microscopy, X-ray and CT imaging, immunohistochemistry.
X-ray and CT imaging showed a clearer lesion in the left middle-upper tibia than in the ipsilateral fibula with enlarged ostealleosis and increased inhomogeneously medullary cavity density, irregular thickening of cortical bone, local cortical default at the inner edge, soft tissue swelling around the abnormal bone. Histologically, tumor tissue was located between the bone trabeculae by nodular, linear and plexiform aggregates of oval-to-spindle cells, large mononucleate cells and multinucleate giant cells with prominent nucleoli and abundant granular eosinophilic cytoplasm. Some aggregates had uncentain amount of discernible lumens, either empty or containing few erythrocytes. A concentric arrangement of oval-to-spindle Cells around small-caliber vascular structures together with collagen fiber contributed to a so-called 'onion-skin' arrangement. The background showed a loose mesenchymal stroma formed of some inconspicuous spindle-fibroblast-like cells, stellate-shape mesenchymal cells, a moderate mononuclear inflammatory cell infiltrate and scattered mast cells. Immunophenotype showed the tumor cells and giant cells strongly positive for vimentin. A good many oval-to-spindle cells stained markedly for CD31 and CD34, but weakly for FVIII, while the giant cells are highlighted instead by CD68, occasionally, very few giant cells showed positive focally for FVIII, a-SMA decorated notedly the cells surrounding the endothelium-like cells but weakly positive in some other tumor cells.
GCAB is a rare, locally infiltrative but slow growing neoplastic angiogenesis with unique morphological characteristics during infancy, which may occur not only in the skin, mucosa, subcutis and deep soft tissue but also in the bone.
研究巨细胞血管母细胞瘤（GCAB）的临床病理特征、影像学特点、免疫表型及鉴别诊断。
对1例发生于左胫骨中上段及腓骨的GCAB进行光镜检查、X线及CT影像学检查、免疫组织化学检查。
X线及CT影像学显示，左胫骨中上段病变较同侧腓骨更清晰，骨质增生、髓腔密度不均匀增高、皮质骨不规则增厚、内缘局部皮质缺损、异常骨周围软组织肿胀。组织学上，肿瘤组织位于骨小梁之间，由椭圆形至梭形细胞、大单核细胞及多核巨细胞呈结节状、条索状及丛状聚集，核仁明显，胞质丰富，嗜酸性颗粒状。部分聚集物有数量不等的可见管腔，管腔可呈空泡状或含有少量红细胞。椭圆形至梭形细胞围绕小口径血管结构呈同心圆排列并伴有胶原纤维，形成所谓的“洋葱皮”样排列。背景为疏松的间充质基质，由一些不明显的梭形纤维母细胞样细胞、星状间充质细胞、中等量单核炎性细胞浸润及散在的肥大细胞构成。免疫表型显示肿瘤细胞及巨细胞波形蛋白强阳性。许多椭圆形至梭形细胞CD31及CD34染色明显阳性，但FVIII染色弱阳性，而巨细胞则被CD68突出显示，偶尔极少数巨细胞FVIII局灶性阳性，α-SMA明显标记内皮样细胞周围的细胞，但在其他一些肿瘤细胞中弱阳性。
GCAB是一种罕见的、局部浸润但生长缓慢的婴幼儿期肿瘤性血管生成，具有独特的形态学特征，不仅可发生于皮肤、黏膜、皮下及深部软组织，也可发生于骨。
