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Primary central nervous system T-cell lymphomas (PCNSTL) are rare, the clinical symptoms and radiographic imaging of which are unspecific, and the pathological morphology is antypical, leading to misdiagnosis and delays in treatment. A 45-year-old male patient with diplopia accompanied by numbness and dysarthria was reported in this paper, which was considered as "lymphoma or lymphoproliferative lesions" on magnetic resonance imaging (MRI) while no typical tumor cells in brain biopsy. The clinical symptoms worsened one month later and the reexamined MRI showed that the scope of the lesion was enlarged and the enhancement was more obvious than before, which was still considered as lymphoma or lymphoproliferative lesion. The second biopsy was performed and still no typical tumor lymphocytes were seen. Finally, gene rearrangement was carried out and showed the β and γ chains both present positive mutations in T cell receptor (TCR) gene rearrangement. Combined with cell morphology, immunophenotype and TCR gene rearrangement results, the patient was finally diagnosed as PCNSTL. This article reviewed the clinical symptoms, imaging features, laboratory examinations, pathological characteristics, diagnosis and differential diagnosis of PCNSTL, so as to improve the understanding of this rare disease.
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Objective:To investigate the expression changes at the transcriptional level in normal lung tissues of mice after exposure to heavy ion radiation for different durations at different doses, aiming to provide evidence for exploring sensitive genes of heavy ion radiation, heavy ion radiation effect and the damage mechanism.Methods:Experiments on the temporal kinetics: the whole thorax of mice was irradiated with 14.5Gy carbon-ions and the total RNA of lung tissue was extracted at 3days, 7days, 3 weeks and 24 weeks. In dose-dependent experiment, the total RNA of lung tissue was extracted at 1 week after irradiated with a growing thoracic dose of 0, 7.5, 10.5, 12.5, 14.5, 17.5 and 20Gy. Protein-to-protein interaction (PPI) analysis and gene-ontology biological process enrichment analysis were performed on significant differentially expressed genes (DEGs).Results:A clearly differential expression patterns were observed at 3-day (acute stage), 1-week (subacute stage), 3-week (inflammatory stage) and 24-week (fibrosis stage) following 14.5Gy carbon-ions irradiation. Among those, the 3-day time point was found to be the mostly different from the other time points, whereas the 7-day time point had the highest uniformity with the other time points. Cellular apoptosis was the main type of cell death in normal lung tissues following carbon-ions exposure. The interactive genes of Phlda3, GDF15, Mgmt and Bax were identified as the radiosensitive genes, and Phlda3 was the center ( R=0.76, P<0.001). Conclusion:The findings in this study provide transcriptional insights into the biological mechanism underlying normal lung tissue toxicity induced by carbon-ions.
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Objective@#To analyze the clinicopathological characteristics and prognosis of diffuse midline glioma (DMG) with H3K27M mutation.@*Methods@#Thirty cases of DMG were collected in Guangdong Sanjiu Brain Hospital from October 2016 to May 2018. The patients′ clinicopathological data including age, tumor site and histological grade, treatment and follow-up data were collected and analyzed.@*Results@#There were 21 males and 9 females, with a mean age of 26 years (range 5-53 years). Fourteen tumors were located in thalamus, 12 in brainstem (one involved both thalamus and brainstem), and one each in hypothalamus, fourth ventricle, and sellar region, respectively. Two cases presented as diffuse intracranial lesions. Three cases (10.0%) were of WHO grade Ⅰ, 10 cases (33.3%) were grade Ⅱ, eight cases (26.7%) were grade Ⅲ, and nine cases (30.0%) were grade Ⅳ.All patients with gradeⅠ tumors were older than 20 years. Histologically, all were pilocytic astrocytoma-like. Immunohistochemical staining demonstrated that all tumors were IDH1 negative. Twenty-eight tumors showed diffuse expression of H3K27M, and two showed focal expression. Twenty-one tumors(100.0%, 21/21) showed absent expression of H3K27me3. Sixteen tumors (57.1%, 16/28) showed strongly positive expression of p53, and ATRX was negative in eight tumors (38.1%, 8/21). The Ki-67 proliferation index ranged from 5% to 40%. Eight cases (including two cases of H3K27M expression of individual cells) showed K27M mutation in H3F3A gene. Intracranial and spinal cord dissemination occurred in six cases (20.0%, 6/30). Median progression-free survival (PFS) was 9.5 months and median overall survival (OS) was 34 months. Mean PFS was 11.2 months and mean OS was 24.3 months. Compared with adults (>20 years old), children/adolescents (no more than 20 years old) had significantly shorter median OS (8 months vs. 34 months, P=0.013). There was no significant difference in PFS and OS between DMGs located in the brain stem/thalamus and other sites within midline (P>0.05). There was no significant difference in PFS and OS between WHO grade ⅠDMGs and WHO grade Ⅱ-Ⅳ DMGs (P>0.05).@*Conclusions@#DMGs occur more commonly in children and adolescents with male predominance. DMGs present with WHO Ⅰ-Ⅳ tumors morphologically, and pilocytic astrocytoma-like lesions with WHO Ⅰ are more common in adults. Expression of H3K27M but not H3K27me3 is helpful for diagnosis of DMG. The prognosis of children/adolescents is significantly worse than that of adults, whereas histological grade and tumor location do not affect prognosis.
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Objective:To retrospectively summarize the clinicopathological features of epithelioid glioblastoma (Ep-GBM) and to explore new treatment for Ep-GBM.Methods:The clinical data of 13 patients with Ep-GBM,who were treated in our department from March 2016 to July 2017,were retrospectively analyzed.The clinicopathological features were summarized and the efficacy was evaluated.Results:The positive rate of BRAFV600E mutant and INI-1 was 76.9％ (10/13) and 80％ (8/10),respectively,while the median Ki-67 index was 30％.Meningeal metastases occurred in 9 cases (69.7％) during the course.The median follow-up time was 12 (6-25) months,and the median progression-free time was 8.6 (2.2-16.5) months.Three patients died and the 1-year overall survival rate was 54％.Conclusion:Ep-GBM has a high degree of malignancy and is prone to spread to leptomeninges.INI-1 expression and BRAFV600E mutation are common for Ep-GBM.BRAF inhibitor might be a potential therapeutic drug for it.
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Objective To assess the impact of additional cycles of temozolomide on the survival of glio-blastoma(GBM)patients after 6 months of maintenance temozolomide(TMZ)following concurrent TMZ chemo-therapy and radiation therapy. Methods Data of 51 GBM patients from 2009 to 2015 were retrospectively studied and the therapeutic effect was assessed according to whether receiving long-term treatment with TMZ. Results Sev-enteen of fifty-one GBM patients received 8 or more cycles and prolonged treatment improved progression-free sur-vival(P=0.011)and overall survival(P=0.004). Conclusions Extended use of TMZ is safe to GBM patients , which may improve response OS and PFS compared to conventional regimen. Prospective studies in larger popula-tions are needed to better-define the population to whom it can be proposed and its optimal duration.
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Objective To study the application of fusion of CT and MRI images in X-knife treatment for intracranial lesions.Methods Total 25 patients included:3 gliomas,3 acoustic neuroma,2 pituitary adenoma,one craniopharygioma to be remained or recurred postoperatily,2 pituitary micro-adenoma,5 AVM,4 cavernous angioma,3 metastatic tumour,one neoplasm located pituitary stem and midbrain respectively.Before the fusion of CT and MRI images MRI scan and CT scan for location of X-knife were performed respectively,then MR and CT image were transferred to workstation for the fusion of images.Results All lesions were showed clearly on fusional images and more nodules were observed in 3 metastatic tumour.Skull,soft tissue constructures and the profile of lesions were completely overlaped on overlaped images of CT and MR with an error less 1.0 mm.Conclusion CT and MRI images of head can be accurately registered.The images can show the radiologic informations more clearly than conventional CT image.It provides a safe,effective and little harmful method for X-knife treatment of intracranial lesions.