| Objective:The clinicopathological and molecular features and prognostic factors in patients with primary central nervous system lymphoma(PCNSL)were analyzed.Methods:1.Collect the clinical data(including treatment plan)and tissue specimens of 35 PCNSL patients in Shanxi Provincial People’s Hospital from 2013 to 2021.2.The molecular expression of CD10,BCL6,MUM1,and Ki-67 were detected by immunohistochemistry.3.Second-generation sequencing technology was used to detect lymphoma-related gene mutations,while comparing the survival differences among different gene mutation groups.4.Survival curves were plotted to analyze the overall survival of 35 PCNSL patients and the impact of different treatment options on survival.5.Clinical characteristics such as age,focal site,lactate dehydrogenase(LDH),immunohistochemistry,and different treatment options were summarized and their impact on prognosis were analyzed.Results:1.Clinical features:1.1.General characteristics: Among the 35 PCNSL patients,42.9%(15/35)were male and 57.1%(20/35)were female.≤60 years 45.7%(16/35),> 60 years 54.3%(19/35),median age of 63 years(17-77).LDH was normal in 34.3%(12/35),elevated in 65.7%(23/35).The lesion invaded the supratentorial tissue 80.0%(28/35),infratentorial tissue20.0 %(7/35).1.2.Clinical manifestations: 17.1%(6/35)of patients had cranial hypertension(headache,dizziness,nausea,vomiting,optic papilloedema),31.4%(11/35)had focal neurological deficit(motor sensory disorders such as convulsions,numbness,weakness,adverse speech,etc.),8.6%(3/35)had neuropsychiatric symptoms(cognitive impairment such as memory impairment,disorientation,calculation impairment,personality disorder,consciousness disorder),42.9%(15/35)showed mixed symptoms.2.Immunohistochemistry results:The pathological types of all the 35 patients were DLBCL.According to Hans subtyping,28.6%(10/35)were of germinal center origin(CD10 was positive or only Bcl-6 was positive),and 71.4%(25/35)were of non-germinal center origin.3.Gene mutation results and effects on survival(second-generation gene sequencing testing was performed in 18 patients):The most common gene mutation was MYD88(66.7%,12/18),IGLL5(38.9%,7/18),PIM1(38.9%,7/18).OS of PIM1 mutation or IGLL5 mutation was worse than the unmutation group,with significant statistical differences(P-values were 0.019 and 0.021,respectively).4.Survival analysis:4.1.Overall survival analysis: The 3-year OS of 35 PCNSL patients were about41.2%.4.2.Effect of treatment regimen on survival: The OS of the high-dose methotrexate(HD-MTX)and rituximab(R)group was significantly better than the untreated group,with statistical differences(P-values were 0.005 and <0.001,respectively).a trend of survival benefit in the Bruton tyrosine kinase inhibitor(BTKi)group,but no statistical difference was shown(P-values were 0.061).5.Prognostic analysis: Univariate and multivariate analysis showed that LDH and different treatment regimens were independent prognostic factors of PCNSL(P-values were 0.007 and 0.002,respectively).Conclusion:1.The PCNSL lesions are mostly located in the supratentorial tissue.Clinically,the most prominent manifestations was the symdroms of damaged brain tissue.Diffuse large b-cell lymphoma,non-germinal center origin,was the mainly pathological type.Elevated serum LDH was significantly associated with poverty prognosis.2.The PCNSL patients have specific genetic alterations,the genes with higher mutation abundance are MYD88,IGLL5,PIM1,and the survival analysis showed that the patients with PIM1 or IGLL5 gene mutations have inferior survival.3.Elevated serum LDH is significantly associated with poor prognosis of PCNSL.MTX and R treatment regimen may improve the survival of PCNSL patients,and,bruton kinase inhibitors for PCNSL are expected to be the first-line treatment regimen. |
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