Dynamic interplay between corticosteroid treatment and the role of SRC-1 gene dysregulation in the progression of WHO-Grade 4 Astrocytoma.

BACKGROUND: Corticosteroid is commonly used before surgery to control cerebral oedema in brain tumours and is frequently continued throughout treatment. Its long-term effect of on the recurrence of WHO-Grade 4 astrocytoma remains controversial. The interaction between corticosteroid, SRC-1 gene and cytotoxic T-cells has never been investigated. METHODS: A retrospective cohort of 36 patients with WHO-Grade 4 astrocytoma were examined for CD8 + T-cell and SRC-1 gene expressions through IHC and qRT-PCR. The impact of corticosteroid on CD8+T-cells infiltration, SRC-1 expression, and tumour recurrence was analyzed. RESULTS: The mean patients age was 47-years, with a male to female ratio 1.2. About 78% [n = 28] of the cases showed reduced or no CD8+T-cell expression while 22% [n = 8] of cases have showed medium to high CD8+T-cell expression. SRC-1 gene was upregulated in 5 cases [14%] and 31 cases [86%] showed SRC-1 downregulation. The average of total days and doses of administered corticosteroid from the preoperative period to the postoperative period was at range of 14-106 days and 41-5028 mg, respectively. There was no significant statistical difference in RFI among tumours expressing high or low CD8+T-cells when corticosteroid was administered in recommended or exceeded doses [p-value = 0.640]. There was a significant statistical difference in RFI between CD8+T-Cell expression and SRC-1 gene dysregulation [p-value = 002]. Tumours with high CD8+T T-cell expression and SRC-1 gene downregulation had late recurrence. CONCLUSIONS: Corticosteroid treatment can directly affect the SRC-1 gene regulation but does not directly influence cytotoxic T-cells infiltration or tumor progression. However, SRC-1 gene downregulation can facilitate late tumor recurrence.

Prognostic value of TP53 expression and MGMT methylation in glioblastoma patients treated with temozolomide combined with other chemotherapies.

OBJECTIVE: To assess the recurrence interval and predictive significance of TP53 expression and O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation in glioblastomas treated with radiotherapy and combined chemotherapies, including temozolomide, lomustine, procarbazine and bevacizumab. METHOD: We reviewed the clinical outcomes of 52 totally resected glioblastoma patients, who received conventional radiotherapy and temozolomide with other chemotherapeutic agents. Correlation of TP53 expression and MGMT promotor methylation with recurrence interval was analyzed using Kaplan Meier estimates. RESULTS: No significant association was found between MGMT promotor methylation and TP53 expression in glioblastomas (P-value = 0.158). Patients with non-methylated MGMT who received temozolomide chemotherapy with other chemotherapeutic agents showed significantly later recurrence (P-value = 0.007) compared with patients with non-methylated MGMT who received temozolomide alone. No significant difference was found in recurrence interval among glioblastoma patients with methylated MGMT who received temozolomide alone or with other chemotherapies (P-value = 0.667). Moreover, patients with non-TP53-expressing tumors who received temozolomide with other chemotherapies had significantly later recurrence (P-value = 0.04) compared with patients who received temozolomide alone. CONCLUSION: Totally resected glioblastoma patients, with non-methylated MGMT or non-TP53-expressing tumors treated with radiotherapy and combined chemotherapies had a reduced chance of tumor recurrence and a more favorable outcome. Furthermore, both MGMT and TP53 are independent prognostic factors for glioblastoma.

Infratentorial immature teratoma of congenital origin can be associated with a 20-year survival outcome: a case report and review of literature.

BACKGROUND: Congenital intracranial tumors are very rare and account for less than 2% of all childhood brain tumors. Teratomas constitute about one third to one half of these, predominantly located midline in the supratentorial region. Posterior fossa location rarely occurs and, based on the cases reported in the literature, commonly has a poor prognosis. CASE PRESENTATION: A newborn female, diagnosed prenatally with hydrocephalus, is presented at birth with increasing head circumference and Parinaud's syndrome. Magnetic resonance imaging scans demonstrated a huge posterior fossa tumor with obstructive hydrocephalus. At surgery, through a suboccipital craniotomy, complete excision was achieved of a histological-proven immature teratoma. The infant received adjuvant chemotherapy for 1 year. She had normal neurological development and remained tumor-free through her 20-year follow-up. CONCLUSION: The authors report this rare case of congenital posterior fossa teratoma with long-term outcome, and the literature is reviewed.

Publications pattern of clinical epilepsy research in Saudi Arabia.

OBJECTIVE: To assess the progress in the field clinical epilepsy in Saudi Arabia, by analyzing in depth the research output productivity and publication pattern, and to identify the current situation of epilepsy research and offer solutions. METHODS: Literature search strategy was designed to retrieve accessible articles that are related to epilepsy utilizing PubMed, Google Scholar, and Embase. The retrieved articles were analyzed with several parameters, then evaluated using Oxford Center of Evidence Based Medicine level of evidence scale. RESULTS: Of all identified articles, 90 were conducted in Kingdom of Saudi Arabia and therefore were included. The included articles had a frequency of only 3.5 publications per year, and growth of 24.4% between the periods of 1990-2003 and 2004-2016. Only 13.3% of the articles were related to surgical epilepsy but the majority (86.7%) were related to medical epilepsy. Many articles (53.3%) were level III studies. The most common study design was retrospective studies in 35.6%, and the citations number ranged from 1-289 (Mean=7). CONCLUSION: Pattern of publications in clinical epilepsy researches revealed a slow growth rate in the frequency and a lower significance in the quality throughout the past 26 years. Active institutional and national promotion of clinical research is needed to help assess and therefore improve the quality of the provided epilepsy services.

The effect of p53 on the activity of NRF2 and NDRG2 genes through apoptotic pathway in IDH-wildtype glioblastoma.

BACKGROUND: Tumor suppressor (p53) acts to integrate multiple stress signals into diverse antiproliferative responses. Its potential to transactivate or downregulate genes through apoptotic pathway in IDH-wildtype glioblastoma has never been explored. METHODS: A group of twenty patients diagnosed with IDH-wildtype glioblastoma, were tested for p53 expression and NDRG2/NRF2 genes activity through protein and gene profiling assays. The connotation between these elements has been explored. RESULTS: The mean patients' age was 64-years. All tumors were IDH-wildtype. p53 was expressed in 12 tumors and absent in 8 tumors. The activity of NDRG2 gene was downregulated in all cases. The activity of NRF2 gene was upregulated in 17 tumors and downregulated in 3 tumors. There was a significant statistical difference in PFS among tumors exhibiting different levels of p53 expression and NDRG2 gene activity [p-value= 0.025], in which 12 tumors with downregulated NDRG2 expression and positive p53 expression had earlier tumor recurrence. This statistical difference in PFS was insignificant when we compared p53 expression with NRF2 gene activity [p-value= 0.079]. CONCLUSIONS: During cell cycle arrest at G2 phase, p53 expression in IDH-wildtype glioblastoma in elderly individuals, coupled with the downregulation of NDRG2 gene activity, led to an aberrant increase in tumor cell proliferation and accelerated tumor recurrence. However, the influence of p53 on NRF2 gene activity was found to be insignificant.

Major Histocompatibility Class-I (MHC-I) downregulation in glioblastoma is a poor prognostic factor but not a predictive indicator for treatment failure.

BACKGROUND: MHC-I expression is a crucial factor in cancer immunity, and its regulations can impact tumor progression and recurrence. The mechanism through which glioblastoma use MHC-I to avoid immunosurveillance has been rarely investigated. METHODS: A retrospective cohort of 35 patients with IDH-mutant WHO-Grade 4 astrocytoma and IDH-wildtype glioblastoma were examined for MHC-I using protein and gene expression assays. The association between IDH mutation, TP53 mutation, and MHC-I expression with recurrence-free interval were investigated. RESULTS: The average patients' age was 49.6 year. IDH was wildtype in 13 tumors. MHC-I protein expression was absent in 30 tumors, faint in 4 tumors, and membrane bound dense expression in single tumor. MHC-I expression was upregulated in 10 tumors and 25 tumors showed MHC-I downregulation. P53 was positively expressed in 19 cases and lost in 13 cases. A significant statistical difference was observed in the RFI between tumors with distinct MHC-I expression and IDH-mutation [p-value = 0.008]. IDH-wildtype tumors with upregulated MHC-I expression showed late tumor recurrence compared to IDH-wildtype tumors with downregulated MHC-I expression. There was insignificant statistical difference in RFI among patients with varying degree of MHC-I expression, who received TMZ or TMZ and other chemotherapies [P-value = 0.44] CONCLUSIONS: Glioblastoma with upregulated MHC-I showed a delayed tumor recurrence in comparison to those with downregulated MHC-I expression. However, downregulated MHC-I may not necessarily be an indicator of poor problems.

Timing of ventriculoatrial shunt removal on renal function recovery of patients with shunt nephritis: Case report and systematic review.

BACKGROUND: Shunt nephritis is autoimmune complex-mediated glomerulonephritis rarely encountered complication following ventriculoatrial shunt (VAS). It's reported to occur after an average of 5.8 years of shunt insertion. We systemically analyzed the literature to know the time of VAS removal that is associated with good renal function recovery. METHODS: We report an unusual case of a 51-year-old female who presented with shunt nephritis 35 years after VAS implantation. This is the longest duration to be reported up to our literature review. A systematic literature review was conducted to assess the factors associated with renal function outcomes in patients with VAS who developed shunt nephritis. RESULTS: Our patient showed a full renal recovery after seven weeks of diagnosis; the atrial catheter was removed, and her shunt was converted to a ventriculopleural shunt (VPLS). Twenty-one articles met the inclusion criteria of our review. Age at shunt insertion of < 3 months is associated with a 66.7% incidence of poor renal outcome (P-value = 0.004). The time from shunt nephritis onset to shunt removal was positively associated with a higher risk of end-stage renal disease or death. A 3-month delay or longer is associated with an odds ratio of 22.4 of poor outcomes (95% confidence interval (CI)=2.2 - 228.7). The time from insertion to nephritis was not significantly associated with the outcome. CONCLUSION: The sooner the shunt is removed within a 3-month interval from the diagnosis of shunt nephritis, the better the outcome, regardless of the time interval from shunt insertion until the development of shunt nephritis.

Optic Pathway-Hypothalamic Glioma Apoplexy: A Report of Two Cases and Systematic Review of the Literature.

Background: Hemorrhage into optic pathway-hypothalamic glioma (OPHG) is rare. Variable clinical presentations and outcomes are associated with such pathology. We aim to present two infants presented with OPHG and a systematic review of the literature. Methods: We describe two cases of infants presenting with sudden decreased vision, poor feeding, and irritability due to OPHG. Both patients underwent urgent craniotomy and subtotal resection followed by chemotherapy. We systematically reviewed the literature using PubMed, Google Scholar, and Embase. In addition, we included all English published reports for all ages discussing the optic pathway (optic nerve and optic chiasm) or hypothalamic glioma associated with hemorrhage from the year of the first reported case (1970) to January 2022. Results: Of 17,949, 44 articles met the inclusion criteria of this review. A total of 56 cases were described with a mean of 21.35 years (0.5-70), with the male gender 52% and the female gender 45%. The hemorrhage location was sellar/suprasellar in 43% cases. Histopathology of included cases was pilocytic astrocytoma in 41%, followed by pilomyxoid astrocytoma in 16% cases. The outcome was unfavorable; 37.5% cases showed improvement, whereas 18% cases resulted in death. Conclusion: Apoplexy of the OPHG can be fatal and associated with poor outcomes. A systematic review of the literature has shown that younger age, pilocytic or pilomexyoid astrocytoma histopathology, and chiasmal/hypothalamic locations are associated with a higher risk of intertumoral hemorrhage and poor prognosis. Further genetic studies for OPHG may provide information for high-risk patients.

Outcome of Anterior and Posterior Endoscopic Procedures for Cervical Radiculopathy Due to Degenerative Disk Disease: A Systematic Review and Meta-Analysis.

STUDY DESIGN: Systematic review and meta-analysis. OBJECTIVES: Cervical spine endoscopic discectomy and decompression have gained popularity in the last decade. This review aimed to shed light on the current outcomes of cervical spine endoscopic procedures for degenerative disc disease (DDD) and to calculate a pooled estimate of various outcome measures. METHODS: We retrieved articles published in English related to endoscopic cervical spine procedures from 3 central databases from inception until September 2020. A subgroup analysis based on the anterior versus the posterior approach was performed. RESULTS: Thirty-one articles fulfilled the eligibility criteria and included 1,410 patients. A successful outcome was observed in 91.3% (88.6-93.4%, P = 0.000). This percentage was lower for the anterior approach (89.6% [85.8-92.5%], P = 0.000) than for the posterior approach (94.2% [90.4-96.5%], P = 0.000). A higher percentage of poor outcomes was reported for the anterior approach (5.7% [3.2-10.1%], P = 0.000 vs. 2.3% [1-5.5%], P = 0.000 for the posterior approach). The overall complication rate was 7.2% (5.2-9.8%, P = 0.000). There was a slightly higher complication rate for the anterior approach (7.9% [4.5-13.3%], P = 0.000) than for the posterior approach (6.7% [4.4-10%], P = 0.000). The revision rate was 4.2% (2.6-6.8%, P = 0.000); and 4.2% (1.8-9.7%, P = 0.000) for the anterior approach and 4.00% (2.2-7.4%, P = 0.000) for the posterior approach. CONCLUSIONS: There is a higher success rate and lower complication rate with the posterior approach than with the anterior approach. However, high-quality randomized controlled trials are vital to evaluate the efficacy of these procedures.

Diagnostic Discrepancies Between Intraoperative Frozen Section and Permanent Histopathological Diagnosis of Brain Tumors.

OBJECTIVE: Intraoperative frozen section (IOFS) diagnosis of brain tumors plays an important role in assessing the adequacy of the sample and determining the treatment plan. The aim of this study was to investigate the diagnostic accuracy between IOFS and permanent sections. MATERIAL AND METHOD: The authors reviewed the histopathological results of 383 brain tumors, including IOFS and permanent histological diagnosis. The cases were classified into three diagnostic compatibilities (i) Perfect fit; the diagnosis of IOFS was identical to the permanent diagnosis, (ii) Partial compatibility; IOFS diagnosis was not incorrect but was too broad to be considered full compatibility, (iii) Conflict; IOFS diagnosis is completely different from the permanent diagnosis. The permanent diagnosis was used as a primary criterion and was compared to IOFS diagnosis and recurrence rate using different statistical methods. RESULTS: 84% of the patients underwent craniotomy and tumor resection, while 15% only underwent tumor biopsy. Approximately, 53.8 % of the cases revealed perfect matching in the diagnosis between IOFSs and permanent sections, while 16.2% of the cases revealed complete mismatching in the diagnosis between the sections. The remaining 30% of the cases showed partial compatibility in the diagnosis between the two diagnostic methods. There was no significant difference in recurrence rate among all cases of different diagnostic compatibility (p=0.54). CONCLUSION: There is a diagnostic discrepancy between IOFSs and permanent sections. However, cases that revealed no consensus in the diagnoses showed no negative effect on the patient outcome. Further studies should be conducted to explore the reasons of this conflict in the two diagnostic methods.

The Diagnostic Value of Pan-Trk Expression to Detect Neurotrophic Tyrosine Receptor Kinase (NTRK) Gene Fusion in CNS Tumours: A Study Using Next-Generation Sequencing Platform.

Background: Neurotrophic tyrosine receptor kinase (NTRK) fusion has been detected in rare types of CNS tumours, which can promote tumorigenesis. The efficacy of Trk inhibitor became a significant therapeutic interest. Our aim was to investigate whether Pan-Trk immunohistochemistry (IHC) is a reliable and efficient marker for detecting NTRK-fusion in different brain tumours. Methods: This study included 23 patients diagnosed with different types of CNS tumours. Testing for Pan-Trk IHC with monoclonal Ab (EPR17341) has been performed on all FFPE tissues. Parallelly, NTRK-rearrangements were tested using both DNA and RNA-based next-generation sequencing (NGS) assay using TruSight Onco500 platform. Results: The cohort included eight pilocytic astrocytomas, one oligodendroglioma, six IDHwildtype glioblastomas, four IDHmutant grade four astrocytomas, and one sample of each (astroblastoma, central neurocytoma, medulloblastoma, and liponeurocytoma). The mean age was 35 years; seven cases were in the paediatric age group, and 16 were adult. Pan-Trk expression was detected in 11 (47.8%) tumours, and 12 (52.1%) tumours showed no Pan-Trk expression. Nine Cases (82%) with different Pan-Trk expressions did not reveal NTRK-rearrangement. The other two positively expressed cases (liponeurocytoma and glioblastoma) were found to have NTRK2-fusions (SLC O 5A1-NTRK2, AGBL4-NTRK2, BEND5-NTRK2). All the 12 cases (100%) with no Pan-Trk expression have shown no NTRK-fusions. There was no statistically significant association between Pan-Trk expression and NTRK-fusion (p = 0.217). The detection of NTRK- fusions using NGS had high specificity over NTRK-fusion detection by using Pan-Trk IHC. Conclusion: Pan-Trk IHC is not a suitable tissue-efficient biomarker to screen for NTRK-fusions in CNS tumours, however RNA-based NGS sequencing should be used as an alternative method.

Immunolocalization of neurokinin 1 receptor in WHO grade 4 astrocytomas, oral squamous cell and urothelial carcinoma.

INTRODUCTION: Neurokinin-1 receptor (NK-1R) induces inflammatory reactions in peripheral tissues but its regulatory effects in target tissues is dependent on receptor signalling. Substance P (SP) has a high affinity for the NK-1R, to which it binds preferentially. We aimed to investigate the expression of NK-1R in World Health Organization (WHO) grade 4 astrocytomas as well as in oral squamous cell carcinoma (OSCC) and urothelial carcinoma, and its association with disease progression. MATERIAL AND METHODS: The study included tissue samples from 19 brain astrocytomas, 40 OSCCs and 10 urothelial carcinomas. NK-1R expression was quantitatively assessed in the tumour cells using immunohistochemistry. The relationship between NK-1R expression in astrocytomas and recurrence-free interval has been explored. RESULTS: The results showed that the NK-1R was intensely expressed in patients with WHO grade 4 astrocytoma, OSCC and urothelial carcinoma. However, cases clinically diagnosed as a low-grade cancer showed reduced NK-1R expression. CONCLUSIONS: NK-1R is overexpressed in all cases of WHO grade 4 astrocytoma, OSCC and urothelial carcinoma. The ubi-quitous presence of SP/NK-1R complex during tumour development and progression suggests a possible therapeutic key strategy to use NK-1R antagonist as an adjuvant therapy in the future.

Acute Hemorrhagic Myelitis in an Adolescent With COVID-19: A Case Report and Review of Literature.

Coronavirus disease 2019 (COVID-19) infection is considered a multisystem disease rather than solely affecting the respiratory system. In addition, many reports have described neurological manifestations of this disease; yet reports on spinal cord involvement, especially in pediatrics, are still limited. We describe a case of a 15-year-old male with COVID-19, who presented with sudden paraplegia and urinary incontinence, preceded by a two-day history of fever. Upon clinical and radiological assessment, he was diagnosed with acute hemorrhagic myelitis. A remarkable motor improvement upon a nine-month follow-up was perceived. Our case illustrates that serious complications can arise even though COVID-19 causes milder disease in pediatrics. We advocate for vaccinating the pediatric population to prevent such occurrences.

Adult-type dermatomyositis with secondary lymphoid follicles harbouring reactive B-cells component.

Dermatomyositis (DM) is an immune-mediated inflammatory disease characterized by pathognomic lesions in skin and skeletal muscle including lymphocytic infiltrates. It rarely presents with ectopic lymphoid structures, as other autoimmune and chronic inflammatory diseases. We describe a case of a 47-year-old male, who presented clinically with proximal muscle weakness, skin rash and elevated creatin kinase (CK) levels. The muscle biopsy revealed inflammatory myopathy, with perifascicular pathology, and scattered ectopic lymphoid follicles-like structures harboring reactive B-cells. Clonality analysis of B-cells using polymerase chain reaction ruled out malignant lymphoma. The patient responded favorably to steroid therapy, and his muscle weakness improved. In conclusion, the clinical and histopathologic features of DM can be atypical, and the presence of lymphoid follicles, although rare, is not inevitably linked to an unfavorable prognosis.

Epidemiological distribution of primary central nervous system tumors in the Western Province of Saudi Arabia: a local registry from neuroscience-affiliated centers.

OBJECTIVES: Central nervous system (CNS) tumors are a major and growing global healthcare challenge. Western Saudi Arabia has an inconsistent data registry; therefore, the epidemiology of CNS tumors is unclear across the country. This study is aimed to assemble the epidemiological matrix of CNS tumors in the Western Province of Saudi Arabia. METHODS: A retrospective analysis was performed using clinical data obtained from 3 neuroscience centers in Western Saudi Arabia in the period 2014-2019. The sample size included 663 adult and pediatric cases from the local and expatriate populations diagnosed with CNS tumors. The distributions of age, sex, clinical presentation, tumor location, type of surgery, histological subtype, genetic characteristics, and recurrence rate were explored. RESULTS: The analysis included 500 adult cases and 163 pediatric cases up to 18 years of age with a male-to-female ratio of 1.16. The mean age at diagnosis was 38.0±22.6 years. The supratentorium was the most common location (n=515, 77.7%). Most patients presented with headache (n=298, 44.9%), followed by a focal neurological deficit (19.9%). The most common primary CNS tumor was glioblastoma (n=234, 35.3%), followed by meningioma (n=100, 15.1%). The recurrence rate after surgery was estimated to be 40.9% among all CNS tumors. CONCLUSIONS: This is the first tumor registry of Western Province of Saudi Arabia that describes the distribution of primary CNS tumors and highlights their epidemiological matrix. Several incidence trends in terms of histological type, age group, sex, location, and recurrence were determined, and some genetic characteristics were recognized.

The Impact of IDH1 Mutation and MGMT Promoter Methylation on Recurrence-Free Interval in Glioblastoma Patients Treated With Radiotherapy and Chemotherapeutic Agents.

The aim of this study is to investigate the relationship between isocitrate dehydrogenase-1 (IDH1) mutation and O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation with recurrence-free interval in glioblastoma patients treated with chemoradiotherapies. Clinical data were collected from 82 patients with totally resected glioblastoma and treated with adjuvant therapies from 2014 to 2019. IDH1 mutation was assessed by immunohistochemistry and MGMT promoter methylation was assessed by different sequencing methods. IDH1 mutation was present in 32 cases and 50 cases were IDH1 wildtype; 54 and 28 patients had unmethylated and methylated MGMT promoter, respectively, Of the 82 patients, 62 patients received chemoradiotherapy while 20 patients only received radiation. Approximately, 61% of patients had a tumor recurrence after 1 year, and 39% showed a recurrence before 1 year of treatment. There was no significant relationship between IDH1 mutation and MGMT promoter methylation (p-value = 0.972). Patients with IDH1 mutation and their age <50 years showed a significant difference in recurrence-free interval (p-value = 0.014). Difference in recurrence-free interval was also statistically observed in patients with unmethylated MGMT promoter and treated with chemoradiotherapies (p-value = 0.031), by which they showed a late tumor recurrence (p-value = 0.016). This revealed that IDH1 mutation and MGMT methylation are independent prognostic factors in glioblastoma. Although IDH1-mutant glioblastomas showed late tumor recurrence in patients less than 50 years old, the type of treatment modalities may not show additional beneficial outcome. Patients with unmethylated MGMT and IDH1 mutation, treated with different chemoradiotherapies, showed a late tumor recurrence.

Sensitivity Assessment of Wilms Tumor Gene (WT1) Expression in Glioblastoma using qPCR and Immunohistochemistry and its Association with IDH1 Mutation and Recurrence Interval.

PURPOSE: Wilms tumor 1 (WT1) gene has recently shown a role in gliomagenesis, making it a potential immunotherapy target in glioblastomas. We aimed to investigate the most sensitive method to detect WT1 expression in glioblastoma and explore the relationship between WT1 expression, IDH1 mutation and recurrence interval. PATIENTS AND METHODS: Clinical data were collected from 44 patients with glioblastomas, treated with adjuvant therapies. WT1 expression was assessed in all cases using immunohistochemistry (IHC), while its gene expression was assessed in 13 clustered samples using polymerase chain reaction (qPCR). IDH1 mutation was assessed using IHC. The sensitivity between IHC and RT-qPCR was examined. Kaplan-Meier curves were used to compare the recurrence-free interval (RFI) between IDH1 and WT1 expression groups. RESULTS: IDH1wildtype was found in 26 cases (59.1%) and the remaining 18 cases (40.9%) were IDH1mutant. Through IHC, WT1 was overexpressed in 32 cases (72.7%), partially expressed in 9 cases (20.5%) and not expressed in only 3 cases. For the 13 cases tested by qPCR, 6 cases showed WT1 upregulation and 7 cases showed WT1 downregulation. There was no significant difference in WT1 expression among cases with different RNA concentrations regardless the testing method (p-value >0.05). However, the difference between IHC and qPCR was significant. IDH1mutant cases with WT1 overexpression showed significant difference in RFI (p-value =0.048). CONCLUSION: Parallel testing for WT1 expression using IHC and qPCR is not reliable. However, IHC provides more accurate results. Moreover, IDH1mutant glioblastomas with WT1 overexpression are associated with late RFI particularly if temozolomide with additional chemotherapies are used.

Long-Term Outcome of Spinal Extranodal Rosai-Dorfman Disease: A Report of Two Cases and Systematic Review.

BACKGROUND: Rosai-Dorfman disease (RDD) is a rare pathologic entity caused by sinus histiocytosis with massive cervical lymphadenopathy. Isolated spinal involvement is an infrequent presentation of extranodal RDD. The clinical and radiologic appearance of RDD represents a diagnostic challenge. We report 2 patients with paraparesis caused by RDD of the thoracic spine and a PRISMA-style systematic review. CASE DESCRIPTION: There were 2 patients with isolated extranodal thoracic spinal RDD without cervical lymphadenopathy. One patient presented with anterior thoracic RDD and a subtotal resection. The small residual disease completely responded to the postoperative course of steroids. The second patient had extradural thoracic spine RDD, which was resected completely. A 6-month postoperative follow-up magnetic resonance imaging (MRI) scan showed local recurrence, which responded to radiation therapy. Five years follow-up of both patients showed normal neurologic functions and no recurrence on MRI scan surveillance. CONCLUSIONS: RDD is a rare occurrence and should be considered in the differential diagnosis of extradural or intradural spinal lesions. Gross total resection is recommended, and long-term clinical follow-up with MRI is advised. Residual or recurrent RDD requires steroids or radiation therapy.

Factors affecting Survival in Glioblastoma: A 10-year Single-Center Experience from Saudi Arabia.

BACKGROUND: Glioblastoma is one of the most common and aggressive brain tumors in adults, which is associated with poor survival rate. This study aims to identify the clinical characteristics and outcome of glioblastoma patients who underwent different treatment modalities and to determine the predictors of survival in them. METHODS: A retrospective chart review conducted at King Abdulaziz University Hospital (KAUH). All patients diagnosed histopathologically with glioblastoma, treated between January 2005 and December 2015, were included. The overall survival rate was calculated using the Kaplan-Mayer method. A univariate analysis was carried out using a log-rank test, and the chi-square test was utilized for categorical data. RESULTS: Thirty-seven patients were included in this study. Age ranged from 5-88 years. 54.1% of the included population were female. Based on immediate postoperative MRI studies, gross total resection was achieved in 40.5%, subtotal resection in 37.8%, and 21.6% underwent biopsy. The majority of patients received adjuvant radiotherapy (56.8%), while 32.5% received adjuvant chemotherapy. The median overall survival was 8.27 months. CONCLUSION: Obtained results are consistent with international published reports. Factors that were associated with poor survival were age <50 years, presenting with signs and symptoms of increased intracranial pressure, postoperative KPS >50, and undergoing biopsy.

Spinal cord compression secondary to idiopathic thoracic epidural lipomatosis in an adolescent: A case report and review of literature.

INTRODUCTION: Spinal epidural lipomatosis (SEL) is a rare condition that presents with progressive spinal cord or nerve root compression. It is commonly reported in patients receiving long-term exogenous steroid therapy or in patients with endogenous steroid overproduction. The occurrence of this condition as an idiopathic entity is rarely reported. CASE PRESENTATION: The authors present the clinical course and outcome of a 16-year-old male student, who presented with progressive spastic paraparesis of a one-year duration caused by idiopathic spinal epidural lipomatosis. Magnetic resonance imaging (MRI) study of the thoracic spine revealed marked compression of the spinal cord from a large dorsally located extradural mass extending from the T-4 to T-12 vertebral bodies. The patient underwent posterior thoracic laminoplasty from the T4 to T10 vertebral levels. He experienced gradual neurological, and he was able to walk without assistant by the end of 3-month follow-up period from surgery. CONCLUSION: Idiopathic SEL is very rare, since no predisposing factors can be identified, and should be included in the differential diagnosis when patients present with spinal neurological compromise. MRI is the imaging modality of choice, and decompressive laminectomy and debulking of the fatty lesion is the main treatment modality in patients with progressive course of the disease..