| Research background and Objective Primitive neuroectodermal tumor(PNET)is a rare small round cell malignancy,which is divided into the central primitive neuroepidermal tumor(c PNET)and the peripheral primitive neuroepidermal tumor(p PNET).In recent years,there have been many breakthroughs in the study of primitive neuroectodermal tumors.So far,CD99 and fli-1 are the most specific immunohistochemical markers for the diagnosis of primitive neuroepidermal tumors.In molecular genetics,the EWSR1 gene rupture is another indicator for the diagnosis of primary neuroectoderm tumors.Peripheral primitive neural ectoderm tumor(p PNET)are relatively rare,and central original neural ectoderm(c PNET)is a rare tumor,in addition they and other small round cell malignant tumor in the histological morphology and immunohistochemical markers have overlap.Therefore,even if the above breakthrough,it is still difficult to diagnose PNET,and easy to misdiagnose.The purpose of this study is: 1.Explore the clinical and pathological features.2.Analyse the charaistics of peripheral primitive neuroectodermal tumors(p PNET)and cal nervous system primitive neurectodermal tumors(c PNET)image.3.The confirmed patient was followed up to analyse of the influence of different factors on patients.4.We hope that this project can improve the understanding of PNET's clinical manifestations,histopathological features,immunohistochemistry and molecular genetics,and reduce misdiagnosis and missed diagnosis.Materials and methods Research object: Collecte a total of 66 primitive neuroectodermal tumors patients diagnosed by surgery or consultation in the first Affiliated Hospital of Zheng Zhou University from August 2011 to December 2017.Method: collect the clinical pathological data and imaging data of these PNET patients.The wax blocks collected were stained with HE and by Immunohistochemical markers CD99,fli-1,CD56,Syn,NSE,Vimentin,CK,Desmin,LCA,Cg A,and myo-d1 with immunohistochemical chlamydia and peroxidase(SP).The wax blocks collected also were used to detect the fracture of EWSR1 gene by the fluorescence original hybridization(FISh method).SPSS17.0 software was used for statistical analysis.the expression differences between CD99 and FLI-1 in p PNET and c PNET were analyzed by chi-square test,and the difference of gene fracture occurred in EWSR1 gene in different age groups of p PNET also were analyzed by chi-square test.Kaplan-meier method was used to carry out single factor survival analysis,and the log-rank method was used to test whether the difference was statistically significant.COX risk regression model was used to carry out multi-factor survival analysis.The test level is 0.05.Results: 1.Clinical features: PNET can occur in various parts of the human body.There were more men than women,with a ratio of 1.4 to 1,ranging from 0.4 to 62 years old,with a middle onset age of 16 years;Among them,59 patients were p PNET,33 males and 26 females,with a ratio of 1.23 to 1.The median age was 16,the youngest was 5 months,and the maximum age was 62.There were 7 cases of c PNET patients,6 males and 1 female,witha ratio of 6 to 1.The median age was 13,the youngest was 5 months,and the maximum age was 29.The prognosis of tumors located in the pelvic or midline areas was worse than that of the non-median tumor(P=0.035).The prognosis of patients with tumor recurrence was worse than that of those without recurrence(P=0.000).The prognosis of patients with preoperative tumor infiltration or metastasis was worse than those without infiltration or metastasis(P=0.005). Gender(P=0.359),age(P=0.222),CD99 expression(P=0.393),and fli-1 expression(P=0.448)had no statistical significance on the survival time.2.Impact performance: p PNET's CT scan was more or less high density.It's MRI is characterized by T1 WI was equal or low signal,T2WI/T2 FLAIR/T2 FS were equal or slightly higher,and the necrosis cyst and hemorrhage were seen in the lesions.DWI was high signal,while in the cystic or necrotic area,the DWI was obviously low signal.c PNET image was given priority to with MRI.Lump was pouch or solid.It often showed the parenchyma of T1 WI was shown as equal or slightly lower signal,T2 WI was equal or high signal,and enhanced examination of the solid part of the tumor showed obvious inhomogeneous enhancement,and peripheral necrosis and cystic parts were not significantly enhanced.3.Pathological features: PNET is a solid mass in the trunk,limbs and brain parenchyma.It has a fish-like cut.PNET in the abdomen is mostly solid-cystic mass.There is a transparent viscous substance in the capsule.The pathological morphology of p PNET and c PNET was similar.In p PNET and c PNET,tumor cells were arranged in clusters,which could be separated into sheets or lobules by interstitial tissue.Tumor cells were small in size,oval or short in shape,not obvious in cytoplasm.Tumor cell nuclei is round or oval?The chromatin is evenly distributed?Nucleolus is not obvious.Tumor necrosis and nuclear fission are common ? there is the Homer-Wright-shaped cluster.4.Immunohistochemical results: In 66 cases of PNET,CD99 positive rate was 92.4%(61/66),the positive rate was 87.9%(58/66),the FLI agreed-1 CD56 positive rate was 63.6%(42/66),the Syn positive rate was 43.9%(29/66),Vimentin positive rate was 71.2%(47/66),NSE positive rate was 43.9%(29/66),and CK,Desmin,LCA,Cg A,Myo-D1 are not expressed in tumor tissues.Among them,59 cases of p PNET,CD99 positive rate was 96.6%(57/59),the positive rate was 88.1%(52/59),the FLI agreed-1 CD56 positive rate was 59.3%(35/59),the Syn positive rate was 44.1%(26/59),Vimentin positive rate was 71.2%(42/59),NSE positive rate was 42.37%(25/59);7 cases c PNET,CD99 positive rate was 57.1%(4/7),positive rate was 85.7%(6/7),the FLI agreed-1 CD56 positive rate was 85.7%(6/7),the Syn positive rate was 42.9%(3/7),Vimentin positive rate was 71.4%(5/7),NSE positive rate was 57.1%(4/7).The positive rate of CD99 in p PNET was higher than the positive rate of CD99 in c PNET,and the difference was statistically significant(P<0.05).The positive rate of fli-1 in p PNET and c PNET was similar,and the difference was not statistically significant(P>0.05).5.Molecular genetic results: A total of 23 cases of p PNET were tested for EWSR1 gene?Among them,There were 20 cases of EWSR1 gene fracture?The minimum onset age was 5 months,the maximum incidence was 61 years,13 males and 10 females,and the ratio of male to female was 1.3:1?A total of 2 cases of p PNET were tested for EWSR1 gene?All of them had a genetic fracture of EWSR1,all male,1 case 5 months,1 case 9 years old?The onset age was all small?In p PNET,the EWSR1 gene was more likely to rupture in younger patients than older adults,and the difference was statistically significant?In p PNET,the EWSR1 gene was more likely to rupture in younger patients than older adults,and the difference was statistically significant.Conclusion: 1.In p PNET,the positive rates of CD99 and FLI1 in tumor tissues were both high,which was significant for the diagnosis of PNET.In c PNET,fli-1 may be more sensitive than CD99.2.EWSR1 gene fracture is a sensitive index for PNET diagnosis.In p PNET,the EWSR1 gene fracture was more likely to occur in younger patients.3.The location of the tumor(The pelvic or midline part),Tumor recurrence,Preoperative tumor infiltration or metastasis were important prognostic factors,It is not related to age,gender,CD99 expression and fli-1 expression. |
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