Clinicopathological features of intracranial primary NTRK-rearranged spindle cell neoplasm
Objective To investigate the clinicopathological features,immunophenotype,molecular biological characteristics,differential diagnosis and prognosis of primary intracranial NTRK-rearranged spindle cell tumors(NTRK-RSCN).Methods The clinical data,pathological features,immunohistochemical markers and molecular biological detection results of a case of NTRK-RSCN were analyzed,and the relevant literature was reviewed.Results The tumor was attached to the left parietal dura and protruded into the brain parenchyma.Microscopically,the tumor was mainly composed of bland spindle cells arranged in fascicular,sheet-like or patternless patterns,with interstitial collagen deposition and focal perivascular hyalinization.Immunohistochemical staining showed that the tumor cells were diffusely positive for Pan-TRK,CD34 and S100,but negative for GFAP,SOX10,SMA and other markers.FISH analysis showed atypical NTRK1 small gap separation signal in the tumor cells.RT-PCR showed that NTRK1 gene had fusion mutation.NTRK1-TPM3 gene fusion was detected by next generation sequencing.Conclusion Primary intracranial NTRK-RSCN is extremely rare and its prognosis is unclear.It should be differentiated from meningioma,solitary fibrous tumor,glioma and so on.