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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 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 investigate the neuroimaging and pathological features of epithelioid glioblastoma (EGBM) to improve the diagnosis.Methods The clinical and pathological features of 4 E-GBM cases were analyzed retrospectively.Results E-GBMs occurred predominantly in young adults.MRI examination showed irregular solidcystic lesion with heterogeneous or ring enhancement in 4 cases.Histological examination revealed uniform population of epithelioid or rhabdoid cells with prominent nucleoli and mitotic activity as well as geographic necrosis..Immunohistochemical staining showed various positive signals of Vimentin and S-100 protein in 4 cases,positive signal of BRAF (VE1) in 3 cases and focal positive signal of GFAP in 1 case.However,IDH-1 was negative and 1p/19q codeletion was lack.All patients were followed-up for 2-6 months.One patient had tumor recurrence 3 months and one patient died of disease 6 months after surgical excision.Conclusion E-GBM has a poor outcome and is closely associated with pleomorphic xanthoastrocytoma.MRI and epithelioid histological features are very important for the differential diagnosis.
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Objective To investigate the neuroimaging and pathological features of epithelioid glioblastoma (EGBM) to improve the diagnosis.Methods The clinical and pathological features of 4 E-GBM cases were analyzed retrospectively.Results E-GBMs occurred predominantly in young adults.MRI examination showed irregular solidcystic lesion with heterogeneous or ring enhancement in 4 cases.Histological examination revealed uniform population of epithelioid or rhabdoid cells with prominent nucleoli and mitotic activity as well as geographic necrosis..Immunohistochemical staining showed various positive signals of Vimentin and S-100 protein in 4 cases,positive signal of BRAF (VE1) in 3 cases and focal positive signal of GFAP in 1 case.However,IDH-1 was negative and 1p/19q codeletion was lack.All patients were followed-up for 2-6 months.One patient had tumor recurrence 3 months and one patient died of disease 6 months after surgical excision.Conclusion E-GBM has a poor outcome and is closely associated with pleomorphic xanthoastrocytoma.MRI and epithelioid histological features are very important for the differential diagnosis.
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Objective To explore the relationship of adult attachment,life orientation style and job burnout among nurses.Methods Relationship Questionnaire (RQ) and Experience of close relationships (ECR) inventory were used to measure 566 nurses' adult attachment.Life orientation test (LOT-R) and Maslach's Burnout Inventory-General Scale (MBI-GS) were administrated to measure subjects'optimism,pessimism,and job burnout.Results ①Distribution of adult attachment style in nurses were 36％ for secure,29.3％ for dismissing,18.4％for preoccupied and 6.7％ for fearful.②Attachment avoidance were positive correlated with pessimism (r =0.139) and negative correlated with optimism (r =-0.212) significantly; and there was significant positive correlation between attachment avoidance and ineffectiveness (r=0.122) also.Attachment anxiety was positively related to pessimism (r=0.151),and three indexes of job burnout (r=0.238,0.281,0.143 respectively).Meanwhile,there were significant negative correlations among optimism and job burnout indexes (r =-0.12,-0.193,-0.230).③There were significant differences among four attachment groups on optimism and pessimism.For job burnout,the four groups had significant differences only on depersonalization,instead of ineffectiveness and emotion exhaustion.④ Regression analysis suggested that attachment anxious was a valid positive predictor of job burnout,while optimism was a negative predictor.Conclusion Both adult attachment and life orientation style are contributed to job burnout among nurses.