骨原发巨细胞性血管母细胞瘤1例报道及文献复习  

Primary giant cell hemangioblastoma of the bone:report of one case and review of literature

作　　者：崔文志 王蔚 闫广宁 梁恭博 赖续文 王卓才 CUI Wen-zhi;WANG Wei;YAN Guang-ning;LIANG Gong-bo;LAI Xu-wen;WANG Zhuo-cai(Department of Pathology,General Hospital of Southern Theater Command,Guangzhou 510010,China)

机构地区：[1]中国人民解放军南部战区总医院病理科,广州510010

出　　处：《诊断病理学杂志》2021年第11期908-912,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨巨细胞性血管母细胞瘤(GCAB)的临床病理特征、免疫表型及其诊断和鉴别诊断。方法分析1例原发于胸椎椎体的GCAB的临床和影像学资料,显微镜下观察其组织学形态及免疫组化特点,并复习相关文献。结果患者男性,45岁,以胸背部疼痛伴双下肢麻木为主要症状。PET/CT示第七胸椎椎体及右侧附件大小3.9 cm×2.1 cm×2.1 cm的不规则膨胀性溶骨性骨质破坏伴代谢增高;MRI亦提示T7椎体及右侧附件异常信号,相应椎管狭窄,脊髓受压变形。组织学:肿瘤组织在骨小梁间呈多结节样或丛状生长,边界不清;结节内肿瘤细胞呈卵圆形或短梭形,围绕血管排列呈洋葱皮样结构,多核巨细胞散在分布于其中;肿瘤细胞胞质嗜酸淡染;胞核轻至中等异型,核膜清晰,可见核内染色质粗颗粒,部分细胞可见小核仁。部分区域血管周围梭形肌周皮样细胞增生呈肌周皮细胞瘤样形态;病变周边区域可见大小不等的小血管增生,常围绕一个中等大小的厚壁血管排列,血管内皮细胞呈鞋钉样或鹅卵石样凸向管腔,形成上皮样血管瘤形态,间质疏松水肿,多量淋巴细胞、浆细胞及吞噬色素的组织细胞浸润。免疫组化显示肿瘤细胞Vimentin阳性,CD31、CD34和FLI-1显示肿瘤内小血管增生,多核巨细胞CD68、AACT和Lysozyme阳性。术后6个月,随访无明显复发。结论GCAB为少见的中间性血管源性肿瘤,具有局部侵袭性的潜在恶性特征。发生于成人骨组织者罕见。临床以手术切除病灶并辅助干扰素治疗为主,效果良好,复发或转移者罕见。Objective To investigate the clinicopathological characteristics,immunophenotype,diagnosis and differential diagnosis of giant cell hemangioblastoma(GCAB).Methods The clinicaland radiation data were analyzed in a case of GCAB originating in the thoracic vertebra.The histological morphology and immunohistochemical characteristics were observed under a microscope,and the relevant literature was reviewed.Results The patient was a 45-year-old male with chest and back pain,numbness in both lower limbs as the main symptoms.PET/CT showed a 3.9 cm×2.1 cm×2.1 cm osteolytic bone destruction with increased metabolism in the seventh thoracic vertebra;MRI also revealed abnormal signals of the 7 th thoracic vertebra and accessories.Microscopically,the tumor tissue grew in multiple nodules or clusters between the trabecular bones,without clear boundary.The oval or short spindle-shaped tumor cells arranged around blood vessels in onion skin-like structure,and multinucleated giant cells scattered among them.The tumor cells was lightly eosinophilic.The nucleus presented mild-moderate atypia,with clear nuclear membrane,coarse chromatin and small nucleoli in some tumor cells.In some areas,spindle-shaped peridermoid cells proliferated in a myopericytoma-like morphology.Vessels with different sizes could be seen in the periphery,which often arranged around a medium-sized thick-walled vessel.Vascular endothelial cells protruded into the lumen with nail-like or cobblestones simulating the epithelioid hemangioma.Lymphocytes,plasma cells and macrophages that phagocytosed pigments were abundant in the edematous stroma.Immunohistochemistry showed that tumor cells were positive for vimentin.CD31,CD34 and FLI-1 showed the proliferation of small blood vessels among the tumor.Multinucleated giant cells were positive for CD68,AACT and lysozyme.During follow-up of six months after resection,there was no recurrence.Conclusion GCAB is a rare intermediate angiogenic tumor with locally aggressive and potentially malignant features.It is rare to occ

关 键 词：巨细胞性血管母细胞瘤 上皮样血管瘤 诊断 鉴别诊断 

分 类 号：R732.2[医药卫生—肿瘤]