骨原发性假肌源性/上皮样肉瘤样血管内皮瘤4例临床病理观察

Pseudomyogenic/epithelioid sarcoma-like haemangioendothelioma of bone: clinicopathological features of four cases

目的 探讨骨原发性假肌源性/上皮样肉瘤样血管内皮瘤的临床病理特征。方法 回顾性分析4例骨原发性假肌源性/上皮样肉瘤样血管内皮瘤的临床资料、病理学形态及免疫组化表型等并复习相关文献。结果 骨原发性假肌源性/上皮样肉瘤样血管内皮瘤好发于青少年,有分布于同侧肢体多处骨的趋势,多见于下肢,镜下包含梭形细胞和上皮样细胞两种成分,伴有以中性粒细胞为主的炎性背景。瘤细胞表达AE1/AE3、FLI-1及FOSB,CD31呈散在特征线状膜表达。结论 骨内原发性假肌源性/上皮样肉瘤样血管内皮瘤是一种中间性偶有转移的血管源性肿瘤,掌握其特征性的组织学形态及免疫表型可作出正确诊断。治疗方式有扩大截骨、化疗、靶向治疗等,需长期随访。

Objective To investigate the clinicopathologic features of pseudomyogenic/epithelioid sarcoma-like haemangioendothelioma of bone(PHE/ES-H). Methods The clinical data, pathologic changes and immunophenotypes were retrospectively analyzed in 4 cases of PHE/ES-H and the related literatures were reviewed. Results PHE/ES-H of the bone was a rare neoplasm, which arised primarily in adolescents. The tumor was tended to be distributed in multiple bones of the ipsilateral limbs, mostly in the lower extremities. Microscopically, it contained two components: spindle cells and epithelioid cells, accompanied by an inflammatory background. The tumor cells expressed AE1/AE3, FLI-1 and FOSB, and CD31 scattered in the characteristic linear membrane. Conclusion PHE/ES-H of the bone is an intermediate occasional metastatic angiogenic tumor, which can be diagnosed correctly by mastering its characteristic histological morphology and immunohistochemical phenotype. The treatment includes extended osteotomy, chemotherapy, targeted therapy and so on, and it needs long-term follow-up.