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目的探讨骨卡波西样血管内皮细胞瘤的临床病理特征及鉴别诊断。方法对1例骨卡波西样血管内皮细胞瘤临床病理特征、免疫表型进行回顾性分析,并复习相关文献。结果患者男性,6岁。CT和MRI示L2-S3椎体多发骨质破坏。临床表现无凝血机制障碍和血小板减少症(Kasabach-Merritt综合征)。镜检见肿瘤由大小不等的小叶结构组成,这些小叶以不规则形式向周围组织浸润,小叶间有纤维性间隔。肿瘤小叶由圆形、卵圆形和短束状排列的梭形细胞、裂隙状血管腔、充血毛细血管和散在其中的纤维素性血栓构成,炎性细胞稀少,核分裂象罕见。免疫组化:肿瘤内皮细胞CD31、CD34和Fli-1(+),D2-40和SMA局灶(+),GLUT-1(-),Ki-67增殖指数1%。结论骨卡波西样血管内皮细胞瘤是一种发生在骨的罕见的血管源性肿瘤,少数病例可局部复发,确切的生物学行为需更多病例的长期随访。
Objective To investigate the clinicopathologic features and differential diagnosis of bone-camberi-like hemangiopericytoma. Methods A retrospective analysis was performed on the clinicopathologic features and immunophenotypes of 1 case of bone camberi-like hemangiopericytoma and the related literature was reviewed. Results The patient was male, 6 years old. CT and MRI showed multiple L2-S3 vertebral bone destruction. Clinical manifestations No coagulopathies and thrombocytopenia (Kasabach-Merritt syndrome). Microscopically, the tumor consisted of lobular structures of varying sizes that infiltrated the surrounding tissue in an irregular pattern with fibrous septa. Tumor lobular by the round, oval and short bundles of spindle cells, fissural cavities, congestion of blood vessels and scattered in which the formation of fibrinous thrombus, inflammatory cells sparse, mitotic rare. Immunohistochemistry: CD31, CD34 and Fli-1 (+), D2-40 and SMA focal area (+), GLUT-1 (-) and Ki-67 proliferation index of tumor endothelial cells were 1% Conclusions Bonewax-like hemangiopericytoma is a rare angiogenic tumor that occurs in bone. A small number of cases may have local recurrence. The exact biological behavior needs more long-term follow-up.