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Objective@#To investigate the clinicopathologic features of intravascular diffuse large B-cell lymphoma (IVLBCL) in the central nervous system (CNS).@*Methods@#The clinical and radiological data of three cases of CNS-IVLBCL in Xuanwu Hospital, Capital Medical University from 2014 to 2017 were collected. And pathological and immunohistochemical features of these patients were retrospectively analyzed. The related literatures were also reviewed.@*Results@#All the three patients aged from 62 to 76 years, with duration of 4-8 months. Clinical manifestations of the three patients included dizziness, fatigue, seizures, etc. They showed intracranial multiple lesions in the frontal, temporal, parietal and corona radiata, etc. MRI demonstrated cerebral infarction or space-occupying lesions. Microscopic observation showed small vessel lumina filled with tumor cells in the white matter. Tumor cells had large, round nucleus, and prominent nucleoli. The chromatin of tumor cells was broadly granular, and the mitotic figures were visible. Tumor cells expressed B cell markers, such as CD20, CD79α, paired box protein 5 (PAX-5). CD34 staining demonstrated that tumor cells were located in the lumen of the blood vessels. Two patients died within half a year after diagnosis, and the other one was lost to follow-up.@*Conclusions@#The clinical symptoms and MRI demonstrations of CNS-IVLBCL are variable and the prognosis is extremely poor. Morphological observation and immunohistochemical phenotyping for biopsy specimens are helpful for early diagnosis and actively combining chemotherapy to prolong survival of patients.
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Objective@#To investigate the expression of LC3B, p-AMPKα and p27 in cortical tuberous sclerosis complex (TSC).@*Methods@#Nineteen specimens of surgically resected TSC cortical tubers were collected at Xuanwu Hospital, Capital Medical University, from 2014 to 2017. The expression of the three proteins in the lesions and the adjacent relatively normal regions was detected by immunohistochemical staining (EnVision two-step method).@*Results@#LC3B was mainly expressed in the dysmorphic neuron and giant cell in TSC cortical tubers and in the adjacent relatively normal neurons, and the expression was diffuse or perinuclear cytoplasmic. There was no significant difference in the average optical density between abnormal cells and neurons adjacent to the lesions (0.343±0.195 vs. 0.419±0.088, P>0.05). p-AMPKα was localized in the cytoplasm of dysmorphic neurons and giant cell in TSC cortical tubers. The average optical density of abnormal cells in the lesions was significantly higher than that of neurons adjacent to the lesions (0.306±0.123 vs. 0.233±0.654, P<0.05). P27 showed nuclear positivity, mainly expressed in the neurons and glial cells close to TSC cortical tubers, while the positive rate in the abnormal cells in TSC cortical tubers was low (15/19 vs. 7/19, P<0.05).@*Conclusion@#There is no significant decrease in the level of autophagy in dysmorphic neurons and giant cells in TSC cortical tubers, which may be related to the compensatory mechanism of AMPK signaling pathway, but without activation of downstream p27.
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Objective To investigate the clinicopathologic features of intravascular diffuse large B?cell lymphoma (IVLBCL) in the central nervous system (CNS). Methods The clinical and radiological data of three cases of CNS?IVLBCL in Xuanwu Hospital, Capital Medical University from 2014 to 2017 were collected. And pathological and immunohistochemical features of these patients were retrospectively analyzed. The related literatures were also reviewed. Results All the three patients aged from 62 to 76 years, with duration of 4-8 months. Clinical manifestations of the three patients included dizziness, fatigue, seizures, etc. They showed intracranial multiple lesions in the frontal, temporal, parietal and corona radiata, etc. MRI demonstrated cerebral infarction or space?occupying lesions. Microscopic observation showed small vessel lumina filled with tumor cells in the white matter. Tumor cells had large, round nucleus, and prominent nucleoli. The chromatin of tumor cells was broadly granular, and the mitotic figures were visible. Tumor cells expressed B cell markers, such as CD20, CD79α, paired box protein 5 (PAX?5). CD34 staining demonstrated that tumor cells were located in the lumen of the blood vessels. Two patients died within half a year after diagnosis, and the other one was lost to follow?up. Conclusions The clinical symptoms and MRI demonstrations of CNS?IVLBCL are variable and the prognosis is extremely poor. Morphological observation and immunohistochemical phenotyping for biopsy specimens are helpful for early diagnosis and actively combining chemotherapy to prolong survival of patients.
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Objective@#To investigate the prognostic impact of alterations of epidermal growth factor receptor(EGFR) and MGMT in glioblastoma.@*Methods@#The retrospective study included 161 supratentorial glioblastomas diagnosed in the Department of Pathology, Xuanwu Hospital, Capital Medical University from 2009 to 2015. EGFR and EGFRvⅢ protein expression was detected by immunohistochemistry; EGFR amplification was detected by fluorescence in situ hybridization; MGMT promoter methylation was detected by pyrosequencing. The change of molecular genetics EGFR and MGMT and outcome were assessed statistically.@*Results@#There were 161 patients, including 85 (52.8%) males and 76 (47.2%) females. The mean age was 53 years, and the median overall survival was 13 months. The integrated classification of glioblastoma included 16 IDH-mutant, 134 wild type, and 11 NOS. The rate of overexpression of EGFR protein was 32.9%(53/161), and that of EGFR amplification was 37.5%(18/48). There was high concordance between immunohistochemistry and FISH(85.4%, Kappa=0.475, P<0.01) and between the level of EGFR protein and EGFR amplification (P<0.01). Twelve cases showed EGFRvⅢ expression, and all also showed EGFR protein overexpression; 149 cases were EGFRv Ⅲ wild type, and EGFR protein overexpression was seen in 27.5%(41/149) of cases. There was no correlation between EGFR and EGFRv Ⅲ expression. Of all cases, 70.2%(106/151) showed MGMT promoter methylation by pyrosequencing. The changes of molecular genetics of EGFR and MGMT were not related. EGFR amplification and protein overexpression had no significant relationship with prognosis. Patients with EGFRv Ⅲ-mutant had shorter survival time than the EGFRv Ⅲ-wild type(P=0.014); patients with MGMT promoter methylation had better prognosis than without (PFS:P=0.002,OS:P=0.006),and MGMT promoter methylation was an independent predictor for overall survival (HR=0.269, 95%CI 0.124-0.583, P=0.001).@*Conclusions@#EGFR protein expression by immunohistochemistry correlates with the status of EGFR amplification. Patients with EGFRv Ⅲ-mutant tumors have poorer prognosis than that with EGFRv Ⅲ-wild type tumors. MGMT promoter methylation is closely associated with prognosis and an independent predictor for overall survival.