黏液型滑膜肉瘤2例并文献复习

发布时间：2018-06-18 01:30

本文选题：软组织肿瘤 + 黏液型滑膜肉瘤　；参考：《临床与实验病理学杂志》2017年02期

【摘要】：目的探讨黏液型滑膜肉瘤(myxoid synovial sarcoma,MSS)的临床病理学特征、诊断及鉴别诊断。方法对2例MSS进行组织形态学观察、免疫组化染色、分子病理学检测并复习相关文献。结果例1男性,例2女性。年龄分别为71岁及15岁。例1肿物位于左下腹,例2肿物位于左侧额颞叶。镜检:例1低倍镜下肿瘤呈结节样,结节内肿瘤由细胞疏松区与致密区组成。疏松区细胞呈漩涡状、束状、片状排列,间质见大量黏液样基质,细胞异型性明显。致密区细胞丰富,瘤细胞呈束状、鱼骨样排列。例2肿瘤由细胞疏松区及致密区组成,两者间相互移行。疏松区细胞呈疏松网状、片状排列,间质见大量黏液样基质。部分区域见少量上皮样细胞,呈小簇状分布。致密区细胞丰富,呈束状、片状排列。未侵犯脑组织。免疫表型:例1,BCL-2、vimentin阳性,α-SMA和EMA部分细胞阳性,CD34、CD57、S-100、CD117、PLAP均阴性。例2,BCL-2阳性,Myo D1胞质阳性,GFAP、Olig-2、EMA、Syn、CD99、Cg A、S-100、Myogenin、STAT6、CD34、desmin、α-SMA均阴性。分子病理学检测:2例均检测到SYT-SSX融合基因。结论 MSS是一种罕见的软组织恶性肿瘤,确诊主要依靠分子病理学检测,临床与病理均需与黏液型纤维肉瘤、孤立性纤维性肿瘤等鉴别。治疗以手术切除联合放疗为主,预后不良。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis and differential diagnosis of myxoid synovial sarcomas. Methods two cases of MSS were observed by histomorphology, immunohistochemical staining, molecular pathology and literature review. Results case 1 male, case 2 female. The ages were 71 and 15 respectively. Case 1 was located in the left lower abdomen and case 2 was located in the left frontotemporal lobe. Case 1 was nodular under low power microscope. The tumor in the nodules was composed of loose and dense areas. In the loose area, the cells were whirlpool, bundles and flakes. A large number of myxoid substrates were found in the interstitial cells, and the heterogeneity of the cells was obvious. Dense area is rich in cells, tumor cells are bundles, fish bone-like arrangement. In case 2, the tumor was composed of loose area and compact area. The cells in the loose area were loose reticular, flaky, and a large number of mucus-like matrix were found in the interstitial. A small number of epithelioid cells were found in some areas and distributed in small clusters. Dense area cells are abundant, bunchy, flaky arrangement. No invasion of brain tissue. Immunophenotype: in case 1, BCL-2 vimentin was positive, and some of the positive cells of 伪 -SMA and EMA were negative for CD34, CD57, S-100, CD117, plap. In case 2, BCL-2 was positive and Myo D1 cytoplasm was positive for GFAPL Olig-2EMAA. The expression of CD 9C 9N C g Agna S-100 and Myogenin statin 6 CD34 desminin, 伪 -SMA were negative. SYT-SSX fusion gene was detected in 2 cases by molecular pathology. Conclusion MSS is a rare malignant tumor of soft tissue. The diagnosis of MSS mainly depends on molecular pathological examination. The clinical and pathological diagnosis should be distinguished from myxofibrosarcoma and solitary fibrous tumor. Surgical resection combined with radiotherapy was the main treatment with poor prognosis.
【作者单位】：福建省晋江市医院病理科;福建省立医院病理科;福建省龙岩市第二医院病理科;
【分类号】：R738.5

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