%0 Journal Article
%T 腹膜后去分化脂肪肉瘤44例临床病理学分析
%A 卢晓　冉雯雯　肖雨婧　张龙宵　何冰　李宏　杨丹丹　李玉军
%J -
%D 2020
%R 10.13362/j.jpmed.202001004
%X 摘要 目的　探讨腹膜后去分化脂肪肉瘤（DDLPS）的临床病理学特征等，旨在为该病患者的临床诊断及预后评估提供依据。 方法　回顾性分析我院2009年1月—2019年11月确诊为腹膜后DDLPS的44例患者的临床资料及肿瘤组织学特征、免疫表型和遗传学特点。并应用免疫组织化学及荧光原位杂交（FISH）方法分别检测CD117、DOG-1、S100、CD34、CD99、SMA、P16、MDM2、CDK4、STAT6、MUC4、MyoD1、Myogenin、β-catenin、ALK的表达和MDM2基因扩增情况。 结果　44例患者发病年龄38～69岁，平均57.5岁，中位年龄59岁；肿瘤最大直径5～50 cm，平均23.4 cm。镜下肿瘤组织中均可见高分化脂肪肉瘤成分，可见散在分布的核大、深染的异型细胞及多核细胞，部分间质黏液性变，可见分支状或弓背状小血管；去分化区与高分化区分布界限清晰，去分化成分的组织学表现呈多样性和异质性，其中多形性未分化肉瘤样16例，高级别黏液性纤维肉瘤样7例，高级别纤维肉瘤样3例，纤维瘤病样5例，低度恶性纤维黏液样肉瘤样3例，孤立性纤维性肿瘤样3例，炎性肌纤维母细胞瘤样1例，骨肉瘤样2例，平滑肌肉瘤样3例，横纹肌肉瘤样1例，3例出现脑膜瘤样漩涡状结节；免疫组织化学示P16、MDM2及CDK4阳性表达率分别为97.7%、93.2%和88.6%；FISH检测示MDM2基因扩增阳性率为95.5%；Log-rank检验显示DDLPS中去分化成分的组织学级别高低与总生存时间无关（P＞0.05），与无进展生存时间也无关（P＞0.05）。 结论　腹膜后DDLPS多发于老年人，肿瘤体积巨大，易复发；免疫组织化学检测P16、MDM2、CDK4和FISH检测MDM2基因扩增具有重要诊断价值；DDLPS中去分化成分的组织学级别高低与术后肿瘤复发及患者生存无关。<br>Abstract：Objective　To investigate the clinicopathological features of retroperitoneal dedifferentiated liposarcoma (DDLPS), and to provide a basis for the clinical diagnosis and prognosis evaluation of this disease. Methods　A retrospective analysis was performed for the data of 44 patients who were diagnosed with retroperitoneal DDLPS in our hospital from January to November 2019, including clinical data, tumor histological features, immunophenotype, and genetic features. Immunohistochemistry was used to measure the expression of CD117, DOG-1, S100, CD34, CD99, SMA, P16, MDM2, CDK4, STAT6, MUC4, MyoD1, Myogenin, β-catenin, and ALK, and fluorescence in situ hybridization (FISH) was used to evaluate MDM2 gene amplification. Results　The age of onset ranged from 38 to 69 years, with a mean age of 57.5 years and a median age of 59 years. The maximum diameter of the tumor ranged from 5-50 cm, with a mean diameter of 23.4 cm. Under the microscope, well-differentiated liposarcoma components were observed in tumor tissue, with scattered enlarged and hyperchromatic heterotypic cells and multinucleated cells, and interstitial mucinous change was observed, as well as branching or arcuate small vessels. There was a clear boundary between the dedifferentiated region and well-differentiated regions, and the dedifferentiated component had diverse and heterogeneous histological features, including undifferentiated pleomorphic sarcoma in 16 patients, high-grade myxofibrosarcoma in 7 patients, high-grade fibrosarcoma in 3 patients, fibroma in 5 patients, low-grade fibromyxoid sarcoma in 3 patients, solitary fibrous tumor in 3 patients, inflammatory myofibroblastoma in 1 patient, osteosarcoma in 2 patients, leiomyosarcoma in 3 patients, rhabdomyosarcoma in 1 patient, and meningioma-like swirled
%K 腹膜后肿瘤
%K 脂肪肉瘤
%K 病理学
%K 免疫组织化学
%K 原位杂交
%K 荧光
%K 细胞遗传学分析
%K 诊断
%K 鉴别
%K 预后<br>Retroperitoneal neoplasms
%K Liposarcoma
%K Pathology
%K Immunohistochemistry
%K In situ hybridization
%K fluorescence
%K Cytogenetic analysis
%K Diagnosis
%K differential
%K Prognosis
%U http://jpmed.qdu.edu.cn/CN/10.13362/j.jpmed.202001004