摘要
目的探讨腹膜后去分化脂肪肉瘤(DDL)的临床病理特征、诊治及预后。方法收集并分析2013年6月至2019年10月收治的15例腹膜后DDL临床病理及影像学资料,并复习相关文献。结果 15例患者中男性13例,女性2例,中位年龄54.3岁。肿瘤平均直径约15.8 cm。12例为原发性病例,3例为继发性病例。CT检查仅7例提示脂肪肉瘤。13例DDL肿瘤完整切除,2例无法完整切除。镜下所见:13例DDL内可见非典型脂肪瘤样肿瘤/高分化脂肪肉瘤(ALT/WDL)成分,2例肿瘤内仅见去分化成分。免疫组化:15例中,Vimentin阳性15例,P16阳性13例,小鼠双微体2(MDM2)阳性14例,CDK4阳性11例,SMA阳性3例,Desmin阳性1例;Ki-67阳性表达率约20%~60%。荧光原位杂交(FISH)法检测:15例DDL均存在MDM2基因扩增。12例获得随访,其中9例复发,复发患者中3例死亡。结论腹膜后DDL对放化疗不敏感,主要采取手术治疗,切除后较易复发,FISH检测MDM2基因扩增情况对诊断DDL具有重要价值。
Objective To explore the clinicopathological characteristics, diagnosis, treatment and prognosis of retroperitoneal dedifferentiated liposarcoma(DDL).Methods The clinicopathological and imaging data of 15 cases of retroperitoneal DDL from June 2013 to October 2019 were collected and analyzed, and the related literature was reviewed.Results Out of 15 cases, 13 were male and 2 were female, with a median age of 54.3 years.The average diameter of tumor was about 15.8 cm.There were 12 cases of primary tumor and 3 cases of secondary tumor.CT scan suggested liposarcoma in only 7 cases.DDL tumors were completely resected in 13 cases and were not completely resected in 2 cases.Pathological examination showed atypical lipomatoid tumor/well differentiated liposarcoma(ALT/WDL) components in 13 cases and only dedifferentiated components in 2 cases.Immunohistochemistry showed that there were 15 cases of Vimentin-positive, 13 cases of P16-positive, 14 cases of murine double minute2(MDM2)-positive, 11 cases of CDK4-positive, 3 cases of SMA-positive and 1 case of Desmin-positive;the positive rate of Ki-67 was about 20%-60%.MDM2 gene amplification was detected by FISH in 15 cases.Of 12 cases followed up, 9 relapsed and 3 of the relapsed cases died.Conclusions Retroperitoneal DDL is not sensitive to radiotherapy and chemotherapy.Surgical treatment is mainly used.It is easy to relapse after surgery.FISH detection of MDM2 gene amplification is of great value in the diagnosis of DDL.
作者
蔡宇翔
张怀念
CAI Yu-xiang;ZHANG Huai-nian(Department of Pathology,Zhongnan Hospital of Wuhan University,Wuhan,Hubei 430071,China)
出处
《中国临床研究》
CAS
2021年第4期513-516,共4页
Chinese Journal of Clinical Research
关键词
去分化脂肪肉瘤
高分化脂肪肉瘤
腹膜后
免疫组化
小鼠双微体2基因
Dedifferentiated liposarcoma
Well-differentiated liposarcoma
Retroperitoneal
Immunohistochemistry
Murine double minute 2 gene
