同时性多中心骨肉瘤一例报道并文献复习

Synchronous multifocal otesosarcoma:a case report and literature review

目的总结并探讨同时性多中心骨肉瘤(synchronous multifocal osteosarcoma,SMOS)的临床特征,探讨其发病机制及治疗与预后。方法报告我院诊治的1例伴有肺转移的SMOS病例;计算机检索2001年至2021年,PubMed、中国知网、万方数据库、维普数据库等公开发表的文献,阅读全文进行二次筛选,回顾分析所有病例的临床特征及预后,探究其发病机制及治疗的研究进展。结果本例病灶≥5处,分别位于左股骨、骨盆、脊柱(T_(1、3),T _(8~9),S_(1))、右锁骨和右第7~9肋骨。采用多药物联合化疗中,随访7个月。现患者肿瘤有扩散趋势,新发数个较小可疑病灶。检索国内外主流数据库近20年内发表的相关文献,纳入27篇文献,166例SMOS病例。患者平均年龄17.39岁,男女比例为1.86∶1。患者确诊时的病灶数目主要为5处及以上(41.3%,43/104),主要病灶最好发于股骨(57.3%,90/157),血清碱性磷酸酶(alkaline phosphatase,AKP)与乳酸脱氢酶(lactate dehydrogenase,LDH)水平普遍升高,血清AKP平均水平为(3468.5±1843.1)U/L,血清LDH平均(1264.9±703.8)U/L。其中放弃治疗者平均生存6个月;仅进行化疗的患者平均生存10.5个月;进行化疗合并放疗的患者平均生存11.3个月;行手术+化疗方案治疗患者的平均生存14.6个月。结论多中心骨肉瘤较为罕见,主要发生于青少年,主要病灶好发于四肢长骨,股骨尤为多见。该病恶性程度高,预后不良,目前SMOS较为有效的治疗为手术联合化疗,但其效果较单灶骨肉瘤差。

Objective To summarize and study the clinical characteristics of synchronous multifocal otesosarcoma,as well as its pathogenesis,treatment and prognosis.Methods A case diagnosed as synchronous multifocal otesosarcoma with lung metastasis was reported.Literature in Pubmed,CNKI,Wanfang database,Weipu database etc.from 2001 to 2021 was searched.The second screening after full-text reading was applied.Clinical characteristics,pathogenesis,treatment and prognosis were explored.Results There were more than 5 lesions located in the left femur,pelvis,spine(T_(1,3),T_(8-9),S_(1)),right clavicle,and right 7-9 th ribs.Multi-agent combination chemotherapy was applied during the follow-up of 7 months.The tumor was still spreading with several new smaller suspicious lesions.We searched databases at home and abroad in the past 20 years for related literature,including27 documents with 166 cases of synchronous multifocal otesosarcoma.The average age was 17.39 years,and the male-to-female ratio was 1.86:1.The number of lesions at the time of diagnosis was mainly 5 or above(41.3%,43/104);lesions mainly located in the the femur(57.3%,90/157);higher serum AKP(3468.5±1843.1)U/L and LDH(1264.9±703.8)U/L.The mean survival time of patients who gave up treatment was 6 months;the mean survival time of patients who received chemotherapy only was 10.5 months;the mean survival time of patients who received chemotherapy combined with radiotherapy was 11.3 months;patients with chemotherapy and surgery had an average survival time of 14.6 months.Conclusions Synchronous multifocal otesosarcoma is rare,but mainly occurs in long bones of the limbs,especially the femur of adolescents.It is highly malignant and the prognosis is poor.Currently,the most effective treatment is surgery combined with chemotherapy,but its effect is much worse than that of single-focal osteosarcoma.