去分化脂肪肉瘤15例临床病理分析  

Dedifferentiated liposarcoma: a clinicopathologic analysis of 15 cases and review of literature

作　　者：涂金花[1] 高德宏[1] 陈培琼[1] 张海萍[1] 钟山[1] TU Jin-hua;GAO De-hong;CHEN Pei-qiong;ZHANG Hai-ping;ZHONG Shan(Department of Pathology,the First Affiliated Hospital of Xiamen University,Xiɑmen 361003,China)

机构地区：[1]厦门大学附属第一医院病理科

出　　处：《诊断病理学杂志》2019年第10期642-645,650,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨去分化脂肪肉瘤(DL)的临床病理特征、诊断及预后。方法对15例DL进行临床病理资料分析、免疫组化及FISH检测,同时对患者进行随访获取预后信息。结果患者年龄29~79岁,中位64岁,男性11例,女性4例;手术标本13例,穿刺标本2例。在13例DL切除标本中,均可见肿瘤由非典型脂肪瘤样肿瘤/高分化脂肪肉瘤(ALT/WDL)和非脂肪性梭形细胞肉瘤组成,11例原发病例(除外2例穿刺标本)中有3例初次取材阅片时未见ALT/WDL成分,经补取材后才发现;2例复发病例未见到高分化脂肪肉瘤成分,均为非脂肪梭形细胞成分。免疫组化:15例DL去分化成分中有10例p16(+),7例MDM2(+),5例SMA(+),4例CD34(+),4例p53(+),3例CD68(+);脂肪肉瘤区域脂母细胞S-100(+);余CD117、DES、STAT6、CK、β-catenin等均为(-)。所有病例均行MDM2检测,FISH结果显示所有病例MDM2基因扩增。去分化区域可以单独或混合呈现以下形态:①纤维肉瘤样(9/15);②炎性肌纤维母细胞瘤样(6/15);③纤维瘤病样(4/15);④多形性未分化肉瘤样(3/15);⑤低度恶性黏液纤维肉瘤样(3/15);⑥血管外皮瘤样(1/15);⑦脑膜瘤样漩涡结构及神经样结构(1/15);⑧乳腺型肌纤维细胞瘤样(1/15);另外还可见软骨及骨分化等。随访期内2例死亡(1例3个月后复发死亡,1例12个月后脑转移死亡),余均存活。结论去分化脂肪肉瘤形态千变万化,分子遗传学MDM2基因检测至关重要;肿瘤发生部位及复发次数是肿瘤预后的关键因素,手术切除是DL主要治疗方法,放、化疗的作用仍有争议,新型靶向治疗是一个潜在治疗方向。Objective To investigate the clinicopathologic features,immunophenotype,genetic characteristics and prognosis of dedifferentiated liposarcoma(DL).Methods 15 cases of DL were analyzed on their clinical and pathological manifestations,immunohistochemistry,fluorescence in situ hybridization(FISH)and follow-up.Results The age of patients ranged from 29 to 79 years(median 64 years).There were 11 males and 4 females.Among 15 cases,2 cases were needle biopsies and 13 cases were surgical specimens.The surgical specimens of DL were composed of atypical lipomatous tumor/well differentiated liposarcoma(ALT/WDL)and non-lipogenic sarcoma.2 relapse cases were composed of non-lipogenc sarcoma only.In the first sampling materials,3 of the 11 primary cases(except 2 needle biopsies)had no atypical lipomatous tumor/well differentiated liposarcoma(ALT/WDL)observed.It was found until materials were resampled in the second time.Immunohistochemical study showed that the dedifferentiated regions expressed P16 in 10 cases,MDM2 in 7 cases,SMA in 5 cases,CD34 in 4 cases,P53 in 4 cases,and CD68 in 3 cases.S100 expression were detected in lipoblast;nevertheless,CD117,DES,STAT6,CK,beta-catenin were negtive in the dedifferentiated areas.The following morphologic features were found in dedifferentiated areas:fibrosarcoma-like(8/15),inflammatory myofibroblastic tumor-like(6/15),fibromatoses-like(4/15),pleomorphic undifferentiated sarcoma-like(3/15),low grade malignant myxfibrosarcoma-like(3/15),haemangiopericytoma-like(1/15),meningothelial like whorling pattern and nerve like structure(1/15)and mammary-type myofibroblastoma(1/15).Besides,heterogeneous components also were found,such as bone,cartilage.2 cases were dead(one was because of multiple recurrences and the other was because of brain metastases),and others was alive during the follow-up period.Conclusions Dedifferentiated areas in DL exhibit a variable histological picture.Molecular genetic testing of MDM2 is crucial to diagnosis of DL.The location and frequency of tumor recurrence are t

关 键 词：脂肪肉瘤 去分化 分子遗传学 预后 

分 类 号：R739[医药卫生—肿瘤]