巨细胞性血管母细胞瘤临床病理观察  

Giant cell angioblastoma: a clinicopathologic analysis

作　　者：余挽澜 陈波 余晓 文学幸 吴蓉 佘高科 

机构地区：[1]常德市第一中医院病理科,湖南常德415000

出　　处：《诊断病理学杂志》2015年第2期91-94,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨巨细胞性血管母细胞瘤(GCAB)的临床病理学特征、影像学特点、免疫组化及诊断。方法对1例26岁女性发生于右前臂、同时累及皮肤、皮下软组织及尺骨中下段的巨细胞性血管母细胞瘤进行影像学、组织学及免疫组化分析,并复习相关文献。结果 X线片显示右尺骨中下段局部骨皮质欠规整,边缘骨质硬化,桡侧骨质不均匀增厚,周围软组织肿胀,未见明显骨膜反应。MRI显示右前臂软组织边界欠清,其内见T1W1等信号,T2W1高、低混杂信号,病灶内信号欠均匀,其长轴与肢体长轴一致,形态欠规则,边界欠清晰;病灶呈匍匐性生长,邻近骨质受侵,皮质欠完整。巨检见右前臂皮肤呈暗红色,表面有紫色颗粒状突起,病变皮肤稍高出正常部位。镜检见骨外软组织病变浸润骨骼肌、脂肪组织,取代骨小梁间的骨髓组织。肿瘤组织呈结节状、梁索状或片状排列,肿瘤细胞呈卵圆形或梭形,部分结节内见多少不等的组织细胞性单核巨细胞、多核巨细胞及含铁血黄素沉积。肿瘤细胞团内见微血管腔和单个细胞胞质空泡,内含单个或多个红细胞;部分结节内肿瘤细胞围绕在微血管周围呈同心圆排列形成洋葱皮样外观。肿瘤间质中见薄壁血窦,大血管壁不规则增厚伴黏液样变性,小血管周边嗜碱性黏液样基质内见多个细胞围成的小腔隙,小腔周围细胞增多彼此相连形成融合趋势,可能是肿瘤细胞团形成的早期形态。肿瘤细胞形态温和,无异型性,未见核分裂及坏死。免疫组化:vimentin弥漫(+),大部分肿瘤细胞CD34和CD31强(+),巨细胞CD68(+);肿瘤细胞聚集区内小血管周围细胞及部分卵圆形细胞、梭形细胞SMA强(+)、calponin弱(+)。结论 GCAB是一种具有独特组织学形态的罕见的先天性血管源性肿瘤,具有局部侵袭性、生长缓慢、惰性生物学行为特点,是一种中间性软组织肿瘤。Objective To explore the clinicopathological features, imaging characteristics, immunophenotypes and diagnosis of giant cell angioblastoma （GCAB）. Methods A case of GCAB, which appeared in the right forearm of a 26- year-old female with skin, subcutaneous tissue and middle-distal ulna involved, was studied by imageology, histology and immunohistoehemistry. Moreover, relevant literatures were reviewed. Results X-ray showed locally irregular cortical bone of the middle and distal ulna, with marginal bone sclerosis, irregular thickening in the radius, soft tissue swelling around the abnormal bone but no obvious periosteal reaction. MRI showed unelear borders of soft tissues of the right forearm, which demonstrated identical T1W1 signal, but mixed intensity T2W1 signal. The signal in the region was uneven and its long axis was paralleled to that of the limb. It had irregular configuration and ill-defined borders. A creeping growth pattern was observed in the lesion. Invasion of adjacent bone and incomplete cortical bone were also seen. In general, the skin of right forearm was dark-red, with purplish granulated processes on the surface. The abnormal skin slightly overtopped the normal site. Microscopically, the lesion in the extraosseous soft tissue infiltrated muscle and adipose tissue, and displaced the bone marrow between the bone trabeculae. Oval-to-spindle tumor cells arranged in nodules, sheets or strips. Some nodules had uncertain amount of histiocytoid mononucleate giant cells, multinucleate giant cells and hemosiderin. In some tumor nodules, there were microvaseular lumens and cytoplasmic vesicles formed in single cell, which contained one or several erythrocytes. In a few nodules, a concentric arrangement of tumor cells around mierovessels formed an ＂onion skin＂ structure. In the stroma of tumor, there were thin-walled sinusoids. Irregular thickening and mucoid degeneration occurred in larger vessel wall. Small lumens formed by several cells was observed in the basophilie myxoid matix around the sma

关 键 词：软组织肿瘤 骨肿瘤 血管母细胞瘤 免疫组化 诊断 

分 类 号：R733[医药卫生—肿瘤]