目的探讨原发性骨腺泡状肉瘤的临床及X线表现,提高对该肿瘤的认识。方法对4例经手术病理证实的骨原发性腺泡状肉瘤患者的临床及X线表现与相关文献进行了回顾性研究。结果肿瘤好发于长管状骨,以下肢多见。主要X线表现为溶骨性和膨胀性骨质破坏,骨膜反应少见,可有软组织肿块。典型的病理组织学特征为肿瘤细胞聚集成实体巢状或腺泡状结构。结论该肿瘤少见,缺乏特征性X线表现,确诊依靠病理学检查。 Objective To investigate the clinical and X-ray findings of primary acinar sarcoma and to improve its understanding of the tumor. Methods The clinical and radiographic features of 4 cases of primary alveolar sarcoma confirmed by surgery and pathology were retrospectively studied. Results tumors occur in the long tubular bone to the lower limbs more common. The main X-ray showed osteolytic and expansive bone destruction, periosteal reaction rare, there may be soft tissue mass. A typical histopathological feature is that tumor cells aggregate into solid nested or acinar structures. Conclusion The tumor is rare, the lack of characteristic X-ray findings, confirmed by pathological examination.