Clinicopathological features of intracranial myxoid mesenchymal tumor with EWSR1 rearrangement
Objective To investigate the clinicopathological characteristics,diagnostic,differential diagnosis,and prognostic features of intracranial myxoid mesenchymal tumor(IMMT)with EWSR1 gene rearrangement.Methods The clinical features and imaging manifestations,histopathology,immunohistochemistry,and molecular detection results of a case of IMMT with EWSR1 gene rearrangement were analyzed.The relevant literature was also reviewed.Results The patient was a 48-year-old male who had been experiencing frequent dizziness for several months without obvious causes.Imaging examination showed a nodular lesion in the right top falx of the brain.Histopathology revealed that the tumor was multinodular,locally cystic,surrounded by lymphoid tissue sleeves and follicular centers,and contained fibrous areas composed of short fusiform cells and significantly mucinous areas in the interstitium.Immunohistochemical staining showed that the tumor cells expressed EMA,CD99,Desmin,and CD68 to varying degrees.Fluorescence in situ hybridization detected EWSR1 gene rearrangement in the tumor cells.The patient was followed up for 32 months after surgery and showed no signs of recurrence or metastasis.Conclusion IMMT with EWSR1 gene rearrangement is a rare type of tumor added to the 2021 World Health Organization classification of central nervous system tumors.The clinical and pathological diagnosis is difficult,which requires a combination of histopathology,immunohistochemistry,and molecular testing.Long-term follow-up shows that these tumors have an indolent biological behavior.