上颌骨纤维结构不良样及骨母细胞瘤样型骨肉瘤临床病理观察

Clinicopathologic features of fibrous dysplasia and osteoblastoma-like osteosarcoma in the maxilla

目的探讨上颌骨纤维结构不良样及骨母细胞瘤样型骨肉瘤的临床病理特征、诊断及鉴别诊断要点。方法对1例上颌骨纤维结构不良样及骨母细胞瘤样型骨肉瘤的临床表现、影像学特征、组织学形态、免疫组化进行分析,并复习相关文献。结果肿瘤由骨纤维结构不良样区域及骨母细胞瘤样骨肉瘤区域组成,瘤细胞异型性小,核分裂少见,瘤组织中见肿瘤性成骨。免疫组化示肿瘤细胞MDM2和CDK 4(+)。结论颌骨纤维结构不良样及骨母细胞瘤样骨肉瘤为低度恶性肿瘤。应与颌骨纤维结构不良、骨母细胞瘤及骨化性纤维瘤相鉴别,MDM2和CDK4二者联合用在其鉴别诊断中具有重要价值。

Objective To investigate the cfinicopathological diagnosis and differential diagnosis of fibrous dysplasia and osteoblastoma-like osteosarcoma of the maxilla （ FDOOM ）. Methods The clinical manifestation, imaging features and pathologic data of FDOOM were analysed. The related literature was reviewed. Results The tumor was composed of fibrious dysplasia-like area and osteoblastoma-like osteosarcoma area, atypical mitotic figures of tumor cells were rare and osteoid matrix of neoplasm could be seen in tumor. Tumor cells were positive for MDM2 and CDK4 by immunohistochemieal staining. Conclusion FDOOM is a low-grade malignant tumors. It should be distinguished from fibrous dyspasia , osteoblastoma and ossifying fibroma. The markers of MDM2 and CDK4 are helpful in differentiation between them.