非长骨骨肉瘤39例临床病理学分析

Clinicopathological analysis of 39 cases of non-long bone osteosarcoma

目的探讨非长骨骨肉瘤的临床病理特征及鉴别诊断。方法采用HE、免疫组化EnVision两步法染色及荧光原位杂交检测39例非长骨骨肉瘤,分析其临床病理特征并复习相关文献。结果非长骨骨肉瘤男女比为14∶25,长骨骨肉瘤男女比为85∶69,两者相比差异有统计学意义(P=0.0333),非长骨骨肉瘤的患者年龄明显高于长骨骨肉瘤。非长骨骨肉瘤发病部位以颌骨(54%)最多见,临床症状多为局部包块伴疼痛,活动受限。影像学主要表现为骨质破坏伴周围软组织肿块影及骨膜反应。病理类型以成骨细胞型骨肉瘤为主。免疫表型及分子检测:患者肿瘤细胞SATB2细胞核均阳性,低级别中央型骨肉瘤CDK4阳性,MDM2阴性;38例普通型骨肉瘤8例CDK4呈阳性;1例MDM2呈弱阳性;FISH检测示低级别中央型骨肉瘤与1例MDM2弱阳性普通型骨肉瘤有MDM2(12q15)基因扩增。非长骨骨肉瘤患者生存分析显示:颌骨骨肉瘤预后良好,肋骨骨肉瘤预后差,其总生存率差异有统计学意义(P=0.0292)。结论非长骨骨肉瘤与长骨骨肉瘤相比,在患者性别、发病年龄及预后均有差异,组织形态学镜下相似,但以成骨细胞型骨肉瘤最常见。诊断需结合临床病史、影像学特征及组织形态学综合判断,并辅以必要的免疫组化或基因检测。

Purpose To investigate the clinicopathological characteristics,and differential diagnosis of non-long bone osteosarcoma(NLBO).Methods 39 cases of non-long bone osteosarcoma were detected by HE,EnVision two-step immunohistochemical staining and fluorescence in situ hybridization.The clinicopathological features of 39 cases were analyzed and the related literatures was reviewed.Results The male to female ratio of NLBO was 14∶25,and that of while long bone osteosarcoma was 85∶69.There was significant difference between the two groups(P=0.0333).In addition,compared with long bone osteosarcoma,the onset age of NLBO was significantly older.The most frequently affected site by NLBO was jaw(54%).Main clinical symptoms of NLBO were local pain and activity limitation.Radiographic features of NLBO were bone destructions with soft tissue shadow and periosteal reaction.The main pathological subtype was osteoblastoma-like osteosarcoma.Immunohistochemistry(IHC)results found that all cases showed tumor nuclear staining for SATB2.Low-grade central osteosarcomas were positive for CDK4 but negative for MDM2.Out of the 38 conventional osteosarcomas,8 were positive for CDK4,and 1 case was weakly positive for MDM2.Fluorescence in situ hybridization(FISH)showed MDM2 amplification in low-grade central osteosarcomas,and 1 conventional osteosarcoma which showed weak positivity for MDM2 in IHC test.There was significant difference in overall survival across the groups of NLBO hat originated from different sites(P=0.0292),with osteosarcoma of the jaw had the best prognosis,whereas osteosarcoma of the rib had the worst prognosis.Conclusion There are significant difference in gender ratio,age of onset,and prognosis between long bone osteosarcoma(LBO)and NLBO.Although NLBO and LBO exhibit similar histomorphological characteristics,osteoblastoma-like osteosarcoma was most common in NLBO.Diagnosis should be combined with clinical history,radiographic characteristics and histopathological features,supplemented by necessary IHC or FISH test.