Myxoid synovial sarcoma: a report of 2 cases and literature review
10.13315/j.cnki.cjcep.2017.02.015
- VernacularTitle:黏液型滑膜肉瘤2例并文献复习
- Author:
Qiuyue LIU
;
Rongjun ZHANG
;
Haijian HUANG
;
Qiuju JIANG
- Keywords:
soft tissue neoplasms;
myxoid synovial sarcoma;
diagnosis;
differential diagnosis;
imunohistochemistry;
molecular pathology
- From:
Chinese Journal of Clinical and Experimental Pathology
2017;33(2):183-185,189
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To investigate the clinicopathological features,diagnosis and differential diagnosis of myxoid synovial sarcoma (MSS).Methods Clinicopathological changes and immunophenotype were retrospectively evaluated in two MSS cases collected from Fujian Provincial Hospital,conbined with genetic mutation analysis.The relevant literatures were reviewed to explore its clinical and pathological features of this tumor.Restilts The two cases,one man and one woman,aged 71 and 15years,respectively.Tumor was located in the left down abdomen in case 1,and left frontal temporal lobe in case 2.Histopathologically,at low magnification in case 1,the tumor was nodular,which was made up of areas of hypercellularity and hypocellularity.In some areas of hypocellularity,the tumor cells were arranged in fascicular,story-form,sheet arrangements with mucoid degeneration.In hypercellularity area,the tumor cells were arranged in fascicular,fish bone-liked arrangenents.At low magnification in case 2,the tumor was nodular,which was made up of areas of hypercellularity and hypocellularity.In hypocellularity area,the tumor cells were arranged in net-like,sheet arrangements,and fascicular,sheet arrangements in case 2.In some area,the tumor cells were epithelioid with cluster distribution,without infringing brain tissue.Immunohistochemically,the tumor cells were diffusely positive for BCL-2,vimentin,and α-SMA and EMA were partially positive,while CD34,CD57,S-100,CD117,PLAP were negative.However,in case 2,only BCL-2 was positive,and MyoD1,GFAP,Olig-2,EMA,Syn,CD99,CgA,S-100,Myogenin,STAT6,CD34,desmin and α-SMA were negative.Molecular detection SYT-SSX fusion gene was detected in both cases.Conclusion MSS is a rare malignancy of soft tissue.The diagnosis of MSS depends on molecular pathology.The clinical and pathological findings are different from mucinous fibrosarcoma and solitary fibrous tumor.The treatment is surgical resection,combined with radiotherapy,with poor prognosis.