Case Of Astrocytoma Research Articles

Risk Stratification in Low Grade Glioma: A Single Institutional Experience.

Low grade gliomas (LGG) are most often noted with the unpredictable overall survival and progression to higher grades. Objective: In the present study, we analyze the clinicopathological features influencing the prognostic outcomes and compared the features with criteria developed by EORTC. We observed the 130 LGG clinical cases in single institute and maintained the follow-up for more than 5 years. In addition, the molecular details were confirmed with markers as IDH, 1p/19q codeletion, p53 and ATRX mutations. The mean age of patients as 37.67 years and male population contributing to 70%. We observed biased incidence among the male population with dominating occurrence at frontal and parietal lobes in the brain. 40.8% patients had oligodendroglioma, 33.8% astrocytoma, 19.2% oligoastrocytoma and 2.3% gemistocytic astrocytoma pathology. Patients who were subjected to chemotherapy and radiotherapy were noted with average survival of 29 months. Oligodendroglial tumors were found with progression free survival (PFS) of 25 months, oligoastrocytoma cases with 32 months, diffuse astrocytoma cases with 23 months while the gemistocytic astrocytoma cases had 22 months. The PFS for LGG cases was 4.7 years while the overall survival was 4.9 years. Mean survival of patients with KPS score <70 and >70 was 1.5 & 4.9 years respectively. 64 patients were observed with the tumor size >5 cm. In total, 72.3% of the patients were underwent GTR, 23.3% STR and 3.8% underwent biopsy. Taken together, the clinical symptoms, expression of molecular markers and the prognosis details provided by our results can help for better management of LGG cases. We further propose to use following five factors to accurately describe the prognosis and tumor recurrence: 1) Age >50 years, 2) tumor size >5 cm, 3) MIB index >5%, 4) KPS score < 70 and 5) gemistocytic pathology.

Clinicopathological associations and prognostic values of IDH1 gene mutation, MGMT gene promoter methylation, and PD-L1 expressions in high-grade glioma treated with standard treatment

Introductionthe objective was to evaluate the impact of IDH1 R132H mutation, MGMT methylation and PD-L1 expression in high grade glioma that received standard therapy (surgery, radiation and chemotherapy) to overall survival (OS).Methodsthis is a retrospective study of 35 high grade glioma cases. Genotyping of IDH1 gene alteration on the mutation hotspot R132 (Sanger sequencing method with Applied Biosystems 3500 Genetic Analyzer), EZ DNA Methylation-Gold kit (Zymo Research) is used to study the methylation, Cell line BT549 (ATCC HTB-122) and HCT-116 (ATCC CCL-247) were used as unmethylated control and partially methylated control respectively. Anti-human PD-L1 antibody clone E1L3N®from Cell Signalling Technology (USA) and Rabbit XP®were used to see PDL-1 expression.Resultsanaplastic astrocytoma cases had more MGMT promoter methylation (50%) than glioblastoma multiforme (GBM) (20%), more IDH1 R132H mutation (42%) than GBM (4.3%). Immunohistochemistry tumor proportion score method (TPS) identified 17% and 8.7% were PD-L1 positive in AA and GBM groups, respectively. Cases with IDH1 R132H mutation and MGMT methylation still showed better OS although with high PD-L1 expression.ConclusionIDH1 R132H mutation and MGMT methylation were good prognostic markers. High expression of PD-L1 apparently might not indicate poor overall survival in the presence of IDH1 R132 mutation and MGMT methylation.

Intracranial Metastases of Intramedullary Spinal Cord Low-Grade Astrocytoma

AbstractThis paper reports a case of intramedullary spinal cord low-grade (LG) astrocytoma that developed brain metastases after 21 months. A 6-year-old child presented with lower spine pain and falls during daily activity. A spinal cord mass was detected using spinal magnetic resonance imaging (MRI), and brain MRI was normal. The spinal lesion was partially resected, and pathological findings revealed LG astrocytoma (WHO Grade II). The patient underwent thoracolumbar radiotherapy. He returned 21 months following initial admission with symptoms of nausea, vomiting, headaches, and seizure. Brain MRI revealed multiple intracranial masses at the posterior fossa, left lateral ventricle, cerebellopontine angles, and left Meckel cave. A recurrent lesion was detected in the thoracic and lumbar regions of the spinal cord and in the cauda equina. The patient underwent chemotherapy. This rare case warns practitioners to monitor closely the cases of spinal cord astrocytoma that are diagnosed as LG tumors based on histology.

Estrogen Receptor Expression in Glial Tumors of Iranian Patients: A Single Center Experience.

Background & Objective:Gliomas are the most common type of primary intracranial tumors in adults. The expression of estrogen receptors varies in different grades of glial tumors, and some studies have suggested that this expression might have a prognostic value. It seems that estrogen receptor expression negatively correlates with the histological grade of gliomas. In the present study, we aimed to determine the expression of estrogen receptor in different glial tumors in Iranian patients and to find a possible correlation between its expression and the grade of glial tumorsMethods:The brain tumors pathology reports from 2014 to 2017 in the Pathology Department of Shohaday-e Tajrish Hospital in Tehran, Iran were evaluated and 104 different gliomas: 79 cases of astrocytoma and 25 cases of oligodendroglioma were selected. All the samples were re-evaluated by a neuropathologist in order to accurately determine the tumor grade. The immunohistochemistry was carried out to detect the expression of estrogen receptor alpha and beta on brain tumors.Results:None of the samples expressed estrogen receptor alpha. In the case of estrogen receptor beta (ERβ), all samples showed various degrees of positivity: 9% weak, 40% moderate, and 51% strong expressions. The level of ERβ expression was found to be conversely correlated with tumor grade.Conclusion:Our study demonstrated that ERβ is expressed in the majority (if not all) of the glial tumors and its expression was conversely related to the tumor grade. Because of well-tolerability and acceptable adverse effects, ER agonists might be considered as therapeutic agents for the patients with glial tumors.

Tumor growth patterns in central nervous system tumors with astrocytic differentiation

Background and Aim: Glial tumors with astrocytic differentiation are the most common primary malignant brain tumors. Hans Joachim Scherer established histological criteria based on hematoxylin and eosin (H&E) staining, which form the basis of the World Health Organization (WHO) glial tumor grades. The aim of this study was to investigate the incidence of Scherer structures across different classes of WHO grade tumors with astrocytic differentiation and determine whether secondary structures can be used as a grade-defining tool. Materials and Methods: Tumor samples were obtained from 36 patients with central nervous system (CNS) tumors with astrocytic differentiation between February 2018 and March 2019. The study was approved by the Committee on Ethics for Scientific Research, Medical University—Varna “Prof. Dr. Paraskev Stoyanov,” Protocol no. 20 [1] on April 26, 2012. The presence or absence of primary Scherer structures (pseudopalisading necrosis, glomeruloid vascular proliferation) and secondary Scherer structures (subpial palisading, fascicular aggregation, satellitosis around neurons, and blood vessels) was analyzed in H&E stained samples. Results: The samples were divided into two groups: 28 glioblastoma multiforme (GBM) cases and 8 lower grade astrocytoma cases. All 28 GBM cases exhibited pseudopalisading necrosis. Glomeruloid vascular proliferation was present only in 89.3% of the GBM cases. The GBM group also showed 67.9% subpial palisading, 78.5% fascicular aggregation of tumor cells, 96.4% perineuronal, and 100% perivascular satellitosis. The lower grade astrocytoma group had 0% pseudopalisading necrosis and glomeruloid vascular proliferation. Among all cases of lower grade gliomas, 50.0% showed subpial palisading, 87.5% fascicular aggregation, 100% perineuronal, and 100% perivascular satellitosis. Conclusions: Secondary Scherer structures can be considered as natural phenomena in glial tumors but cannot be used as features for grading.

NI-15 THE USEFULNESS OF PET IMAGING IN MOLECULAR DIAGNOSIS OF GLIOMA

OBJECTIVEAfter WHO 2016 Classification of Tumors of the Central Nervous System have published, molecular diagnosis became part of the diagnostic criteria. In this study, we investigated the correlation between PET images and molecular diagnosis of glioma.METHODSWe performed retrospective review of newly diagnosed supratentorial glioma patients who preoperatively underwent all four PET examinations (18F-FDG, 11C-MET, 18F-FLT and 18F-FMISO) from April 2009 to March 2019. The standardized uptake value (SUV) from the accumulation of each PET tracers, TNR (tumor to contralateral normal tissue ratio) of 18F-FDG,11C-MET and 18F-FLT, TBR (tumor to blood values ratio) of 18F-FMISO were measured. We investigated the correlation between these PET images and molecular diagnosis of glioma.RESULTSData from total of 79 patients which were 42 cases of IDH wild type glioblastoma, 2 cases of IDH mutated glioblastoma, 9 cases of IDH wild type astrocytoma, 13 cases of IDH mutated astrocytoma and 13 cases of IDH mutated and 1p/19q co-deleted oligodendroglioma were included in this study. Both TNR of 11C-MET(p<0.01) and 18F-FLT(p<0.01), and also TBR of 18F-FMISO(p<0.01) in IDH wild type gliomas showed significantly higher than IDH mutated gliomas. In WHO Gr2-3 gliomas, only TNR of 18F-FLT showed a significant difference between IDH wild type gliomas and IDH mutated gliomas(p<0.01). TNR of 18F-FLT(p<0.01) and TBR of 18F-FMISO(p<0.01) in 1p/19q co-deleted gliomas were significantly lower than gliomas without 1p/19q co-deletion, but there were no significant differences in WHO Gr2-3 gliomas. Among IDH mutated gliomas, TNR of 11C-MET in 1p/19q co-deleted gliomas showed significantly higher uptake than gliomas without 1p/19q co-deletion(p<0.05).CONCLUSIONPreoperative PET evaluation of each PET tracers may be useful for the molecular diagnosis of glioma.

Atypical clinical manifestations and prognosis analysis of pediatric diffuse intrinsic pontine glioma

Objective To analyze the atypical clinical manifestations and clinical prognosis of pediatric diffuse intrinsic pontine glioma (DIPG). Methods A total of 27 patients with complete clinical data of DIPG undergoing clinical treatment at Neurosurgery Department of Beijing Tiantan Hospital, Capital Medical University from November 2015 to November 2017 were retrospectively enrolled into this study. All patients were divided into 2 groups according to the presence of atypical manifestations: atypical clinical manifestation group (17 cases) and the control group (10 cases). Among the 27 cases, 16 patients underwent stereotactic biopsy or open cranial biopsy, and the other 11 patients underwent tumor excision through craniotomy. All the children were followed up clinically or by telephone after operation to study the clinical outcomes. Comparative analysis was conducted on those clinical data. Prognosis was compared between the 2 groups through Kaplan-Meier survival analysis. Results Among the 27 cases, 20 were pathologically identified as high grade gliomas (including 4 cases of diffuse midline glioma, 6 cases of glioblastoma, 6 cases of anaplastic oligoastrocytoma, 4 cases of anaplastic astrocytoma) and 7 as low grade glioma (including 4 cases of oligoastrocytoma and 3 cases of astrocytoma). One child suffered from pneumonia and finally recovered postoperatively. No statistically significant difference (all P>0.05) was identified between 2 groups in the age, gender, clinical manifestation (including cranial nerve palsy, long tract dysfunction and cerebellum signs), clinical imaging features (including lesion enhancement, supratentorial ventricle enlargement and brachium pontis involvement), clinical treatment or pathology. Kaplan-Meier survival analysis showed that patients with atypical clinical manifestation had significantly higher cumulative survival rate than those in the control group (P=0.045). Conclusion Atypical clinical manifestation could be identified in some pediatric DIPG patients, which might be predictive of a better prognosis. Key words: Brainstem tumor; Child; Atypical clinical manifestation

GENE-30. PRELIMINARY STUDY ON WHOLE-EXOME SEQUENCING TECHNOLOGY IN THE GENETIC ANALYSIS OF PEDIATRIC PATIENTS WITH GLIOMAS

Abstract OBJECTIVE To analyze the genes related to the signaling pathways in pediatric gliomas and drug-related genes with whole-exome sequencing technology. METHODS The tumor tissues and matched blood samples of 17 enrolled patients were detected with whole-exome sequencing technology. There were 3 cases of diffuse midline gliomas, 2 cases of childhood glioblastomas, 3 cases of disffuse astrocytoma, 1 case of pleomorphic xanthoastrocytoma, 1 case of ganglioglioma, 6 cases of anaplastic ependymoma and 1 case of ependymoma in this study. All the enrolled patients who were no more than 14 years old received surgery in the Department of Neurosurgery, Guangdong Sanjiu Brain Hospital. The diagnosis was confirmed by pathological examination and the sample acquisition was approved by hospital ethics committee. RESULTS With the use of whole-exome sequencing technology, a total of 31 related genetic mutations were detected in 15 cases, while no genetic mutation was detected in the other 2 cases. The genes related to the signaling pathways in pediatric gliomas included ATRX, ASL1, BCOR, EP300, FGFR1, H3F3A, IGF1R, MED12, PIK3R1, PRKDC, RB1, SETD2, SMARCA4, SOX2, TGFBR2, and the drug-related genes included AKT1, BCL2, BRAF, BRCA2, CCND1, CCND2, CDK6, EGFR, FGF3, KRAS, MET, PDGFRA, PIK3CA, PTEN, TP53, TSC1. One patient only had genes related to the signaling pathways, and 14 patients had drug-related genes. CONCLUSION Applying whole-exome sequencing technology in the genetic analysis of pediatric patients with gliomas has remarkable guiding significance for revealing the mechanism of disease, searching for therapeutic targets and adopting individualized treatment, which can bring potential benefits to pediatric patients. However, more samples and further data analysis and verification are needed in future study.

IDH-1 polymorphisms in pilocytic astrocytomas

As of 2016, isocitrate dehydrogenase (IDH)-1 and IDH-2 mutations are part of the definition of an oligodendroglioma and may be seen in a significant subset of grade II-IV fibrillary astrocytomas. Reports of IDH-1 and IDH-2 alterations in pilocytic astrocytomas have been rare. This study reports two cases of pilocytic astrocytomas which harbored IDH-1 polymorphisms (G105G) (c.315C > T) discovered on polymerase chain reaction (PCR) testing and sequencing. The first was encountered in a 21-year-old male with a right orbital frontal pole mass. The second occurred in a 19-year-old female with a right frontal tumor. Neither tumor stained with antibody to IDH-1 (R132H). No BRAF V600E immunostaining, minimal p53 staining (<5%) and no loss of ATRX staining was noted in both cases. The significance of the IDH-1 findings at this juncture is uncertain. Misdiagnosis of the tumor as a fibrillary astrocytoma or oligodendroglioma due to the presence of an IDH alteration should be avoided.

P04.16 TP53 mutations in codon 273 is predictive of overall survival in astrocytoma patients

Abstract BACKGROUND Tumour Protein 53 (TP53) is a tumour suppressor gene that is mutated in at least 50% of human malignancies. The prevalence of TP53 mutation is much higher in astrocytomas with reports of up to 75% TP53 mutant cases. Rare cases of TP53 mutation also exist in oligodendroglial tumours (10–13%). P53 pathway is therefore an important factor in low-grade glioma tumorigenesis. Although the prognostic impact of TP53 mutations has been studied previously, no concrete concordance were reached between the studies. In this study, we investigated the prognostic effects of TP53 mutation in astrocytoma and oligodendroglioma. MATERIAL AND METHODS A cohort of 65 matched primary and recurrent fresh frozen tumours were sequenced to identify hotspot exons of TP53 mutation. Exons 1 to 10 were sequenced and pathogenic mutations were mostly predominant between Exons 4 and 8. The cohort was further expanded with 78 low grade glioma fresh frozen tissues and hotspot exons were sequenced. Selecting only the primary tumour from 65 matched tumours, a total of 50 Astrocytoma cases and 51 oligodendroglioma cases were analysed for prognostic effects of TP53. Only pathogenic TP53 mutations confirmed through COSMIC and NCBI databases were included in the over survival and progression-free survival analysis. RESULTS 62% (31/50) of astrocytomas and 16% (8/51) of oligodendrogliomas harboured pathogenic TP53 mutations. Pathogenic hotspot mutations in codon 273 (c.817 C&gt;T and c.818 G&gt;A) was prevalent in astrocytoma with 58% (18/31) of tumours with these mutations. TP53 mutation status was maintained between primary and recurrent tumours in 93% of cases. In astrocytoma, overall survival of TP53 mutant patients was longer compared to TP53 wild-type patients (p&lt;0.01) but was not significant after adjusting for age, gender, grade and IDH1 mutation status. In contrast, astrocytoma patients with specific TP53 mutation in codon 273 showed significantly better survival compared to other TP53 mutant and TP53 wild-type patients combined (p&lt;0.01) in our multivariate analysis. Time to first recurrence (progression-free survival) of TP53 mutant patients was significantly longer than TP53 wild-type patients (p&lt;0.01) after adjustments were made, while TP53 mutation in codon 273 was not prognostic for progression-free survival. In oligodendroglioma patients, TP53 mutations did not significantly affect overall survival and progression-free survival. CONCLUSION In agreement with others, TP53 mutation is more prevalent in Astrocytoma and mutations in codon 273 are significantly associated with longer survival.

Medulloblastoma: Clinical presentation

Medulloblastomas present generally a-specifically as a fast-evolving posterior fossa tumor. Medical literature is poor concerning clinical features of medulloblastomas and their potential significance. In the present study, we reviewed 91 pediatric observations of medulloblastomas treated in Lille between 1997 and 2017. Clinical and epidemiological variables were collected and intercorrelated. They were also compared with anatomical and pathological findings, and outcome, with the aim of defining clinical-pathological entities. We also compared the group with 32 cases of posterior fossa ependymoma and 130 cases of cerebellar astrocytoma treated during the same period. We found that in medulloblastomas, the M/F ratio was higher and diagnostic delay was shorter than in astrocytomas. Also, the mean age was older than in ependymomas. Intracranial hypertension was constant; we further observed that altered general status was common (16.5%) and correlated with a metastatic tumor. We delineated two clusters: the “nodular” cluster, which associates young age, cerebello-pontine angle tumor, herniation, desmoplastic tumor, and tumor predisposition syndrome; and the “metastatic” cluster, which associates altered status, initial metastases, hydrocephalus, and diagnostic delay. Meticulous collection of clinical data at the initial phase is integral part of the oncological evaluation, with a search for genetic and prognostic risk factors, which then permits us to define clinical-pathological entities.

Endoscopic Surgery of Third Ventricular Lesions

AbstractBackground: The endoscopic surgery to third ventricular lesions are now widely used as it is less invasive than the traditional microsurgical procedures.Aim of Study: To evaluate endoscopic surgery of third ventricular lesions as regards clinical improvement, compli-cation rate and extent of lesion resection.Patients and Methods: Retrospectively eleven cases of third ventricular lesions (seven cases of colloid cyst, two cases of astrocytoma, one case epidermoid cyst and 1 case of arachnoid cyst) were operated upon through endoscopic route for total excision if feasible, pre-operative assessment in the form of full neurological examination and imaging (CT and MRI) were done for all patients. Incompletely removed lesions were managed by a septostomy and VP shunt, post-operatively CT was performed in the first 24 hours after surgery and another after 48 hours, MRI with contrast was done 6 months post-operatively to detect recurrence, patients with persistent post-operative hydrocephalus had VP shunt insertion.Results: Complete resection was achieved in nine patients (82%), while resection was incomplete in two cases (18%). complications were in one case (9%) in the form of short memory affection improved after one month, post-operative hydrocephalus was in one patient (9%), mean length of hospital stay was three and half days, no recurrence was reported in all patients with complete resection for a follow-up period of 12 months.Conclusion: Endoscopic resection of third ventricular lesions is safe, minimally invasive, technically feasible and with good outcome especially in completely removed lesions.

Radiological Characteristics and Natural History of Adult IDH-Wildtype Astrocytomas with TERT Promoter Mutations.

Adult IDH-wildtype astrocytomas with TERT promoter mutations (TERTp) are associated with a poor prognosis. To analyze the radiological presentation and natural history of adult IDH-wildtype astrocytomas with TERTp. We retrospectively reviewed the characteristics of 40 IDH-wildtype TERTp-mutant astrocytomas (grade II n=19, grade III n=21) and compared them to those of 114 IDH-mutant lower grade gliomas (LGG), of 92 IDH-wildtype TERTp-mutant glioblastomas, and of 15 IDH-wildtype TERTp-wildtype astrocytomas. Most cases of IDH-wildtype TERTp-mutant astrocytomas occurred in patients aged >50 yr (88%) and presented as infiltrative lesions without contrast enhancement (73%) that were localized in the temporal and/or insular lobes (37.5%) or corresponded to a gliomatosis cerebri (43%). Thalamic involvement (33%) and extension to the brainstem (27%) were frequently observed, as was gyriform infiltration (33%). This radiological presentation was different from that of IDH-mutant LGG, IDH-wildtype TERTp-mutant glioblastomas, and IDH-wildtype TERTp-wildtype astrocytomas. Tumor evolution before treatment initiation was assessable in 17 cases. Ten cases demonstrated a rapid growth characterized by the apparition of a ring-like contrast enhancement and/or a median velocity of diametric expansion (VDE) ≥8 mm/yr but 7 cases displayed a slow growth (VDE <8 mm/yr) that could last several years before anaplastic transformation. Median overall survival of IDH-wildtype TERTp-mutant astrocytomas was 27 mo. IDH-wildtype TERTp-mutant astrocytomas typically present as nonenhancing temporo-insular infiltrative lesions or as gliomatosis cerebri in patients aged >50 yr. In the absence of treatment, although rapid tumor growth is frequent, an initial falsely reassuring, slow growth can be observed.

Perioperative Nursing Care Experience of a Case of Postoperative Recurrent Grade II-I Left Frontal Astrocytoma

Objective: To investigate the effect and value of perioperative nursing intervention on postoperative recurrent grade II-I left frontal astrocytoma. Method: Nursing care procedures: left frontal lobe of the patient was occupied by a large space. Clinical teaching of nursing was strengthened. Preoperative, intraoperative and postoperative care and ward patrol were enhanced. Close attention was paid on consciousness, pupil, vital signs, body temperature, lungs condition, cranial and nerve function of the patient. Extra care was taken in keeping the incision clean and dry. Blood diffusion condition in wound was observed. Active cooperation with doctor was taken to prevent occurrence of pulmonary infection, urinary tract infection, pressure ulcer and other complication. Results: After operation, the patient had stable condition, clear mind and consciousness. There was no occurrence of epilepsy but intracranial hemorrhage. Pneumatosis was relieved. There was no infection and other complication. Conclusion: Application of comprehensive nursing intervention could effectively improve quality of life of patient and reduce incidence of postoperative complication in patients with brain tumor.

Clinical, histopathological, and molecular analyses of IDH-wild-type WHO grade II-III gliomas to establish genetic predictors of poor prognosis.

The genetic features of isocitrate dehydrogenase-wild-type (IDH-wt) lower-grade gliomas (LGGs; World Health Organization grades II and III) are not well defined. This study analyzed the genetic and other features of IDH-wt LGGs to develop a subclassification that can be used to predict their prognosis. Clinical, histopathological, and genetic features of 35 cases of diffuse IDH-wt astrocytoma and IDH-wt anaplastic astrocytoma were analyzed. The following genetic factors were examined: mutations of B-rapidly accelerated fibrosarcoma, telomerase reverse transcriptase promoter (TERTp), histone 3 family 3A, and alpha-thalassemia/mental retardation syndrome, X-linked; and copy number aberrations. In the univariate analysis, the following factors were associated with poor overall survival (OS): the histopathological diagnosis, TERTp mutation, the gain of chromosome 7 (+ 7), and the loss of chromosome 10q (- 10q). In the multivariate analysis, + 7, - 10q, and TERTp mutation were independent prognostic factors associated with poor OS. The median OS was significantly worse for patients who harbored at least one of these factors than for those without any of them (18.5 vs. 54.5months, P = 0.002). The subclassification of IDH-wt LGGs according to the genetic factors + 7, - 10q, and TERTp mutation is potentially useful for predicting the prognosis.

Cerebral pilocytic astrocytoma with spontaneous intratumoral haemorrhage in the elderly - a rare entity

Pilocytic astrocytomas (PA) are histologically are low-grade tumours, commonly found in the paediatric and young adult population. Although cases of adult and elderly pilocytic astrocytomas are described in the literature, they are quite uncommon. The rate of PAs with spontaneous bleeding is very less, with a high occurrence in children than in adults, moreover, that is in cerebellar PAs. Cerebral PAs with intratumoral haemorrhage in the older age group is extremely rare. We present a case of a 60-year female presenting with acute neurological compromise resulting from an acutely haemorrhagic Left temporal pilocytic astrocytoma. She was managed surgically with evacuation of tumoral haemorrhages, as well as resection of the tumour. Postoperatively the patient made a remarkable recovery.

Astrocytoma of the optic nerve head

In this article, a clinical case of astrocytoma of optic nerve head in patient with neurofibromatosis type 1 is presented. The main feature of this clinical case is a difficulty in differential diagnosis with amelanotic choroidal melanoma.

MR imaging phenotype correlates with extent of genome-wide copy number abundance in IDH mutant gliomas.

There is variability in survival within IDH mutant gliomas determined by chromosomal events. Copy number variation (CNV) abundance associated with survival in low-grade and IDH mutant astrocytoma has been reported. Our purpose was to correlate the extent of genome-wide CNV abundance in IDH mutant astrocytomas with MRI features. Presurgical MRI and CNV plots derived from Illumina 850k EPIC DNA methylation arrays of 18 cases of WHO grade II-IV IDH mutant astrocytomas were reviewed. IDH mutant astrocytomas were divided into CNV stable group (CNV-S) with ≤ 3 chromosomal gains or losses and lack of focal gene amplifications and CNV unstable group (CNV-U) with > 3 large chromosomal gains/losses and/or focal amplifications. The associations between MR features, relative cerebral blood volume (rCBV), CNV abundance, and time to progression were assessed. Tumor rCBV estimates were obtained using DSC T2* perfusion analysis. There were nine (50%) CNV-S and nine (50%) CNV-U IDH mutant astrocytomas. CNV-U tumors showed larger mean tumor size (P = 0.004) and maximum diameter on FLAIR (P = 0.004) and also demonstrated significantly higher median rCBV than CNV-S tumors (2.62 vs 0.78, P = 0.019). CNV-U tumors tended to have shorter time to progression although without statistical significance (P = 0.393). Larger size/diameter and higher rCBVs were seen associated CNV-U astrocytomas, suggesting a correlation of aggressive imaging phenotype with unstable and aggressive genotype in IDH mutant astrocytomas.

5-ALA fluorescence-guided surgery in pediatric brain tumors-a systematic review.

5-Aminolevulinic acid (5-ALA)-guided resection of gliomas in adults enables better differentiation between tumor and normal brain tissue, allowing a higher degree of resection, and improves patient outcomes. In recent years, several reports have emerged regarding the use of 5-ALA in other brain tumor entities, including pediatric brains tumors. Since gross total resection (GTR) of many brain tumors in children is crucial and the role of 5-ALA-guided resection of these tumors is not clear, we sought to perform a comprehensive literature review on this topic. A systematic literature review of EMBASE and MEDLINE/PubMed databases revealed 19 eligible publications encompassing 175 5-ALA-guided operations on pediatric brain tumors. To prevent bias, publications were revised independently by two authors. We found that 5-ALA-guided resection enabled the surgeons to identify the tumor more easily and was considered helpful mainly in cases of glioblastoma (GBM, 21/27, 78%), anaplastic ependymoma WHO grade III (10/14, 71%), and anaplastic astrocytoma (4/6, 67%). In contrast, cases of pilocytic astrocytomas (PAs) and medulloblastomas 5-ALA-guided surgery did not show consistent fluorescent signals and 5-ALA was considered helpful only in 12% and 22% of cases, respectively. Accumulation of fluorescent porphyrins seems to depend on WHO tumor grading. One important finding is that when 5-ALA-guided resections were considered helpful, the degree of resection was higher than is cases where it was not helpful. The rate of adverse events related to 5-ALA was negligible, especially new postoperative sequelae. 5-ALA could play a role in resection of pediatric brain tumors. However, further prospective clinical trials are needed.

Russian Scientific Center of Roentgenoradiology of the Ministry of Healthcare of the Russian Federation

The development of secondary tumors is a rare but well-known late effect of radiation therapy of lesions of the central nervous system. At the same time, malignant gliomas most often occur. This article presents a rare clinical case of secondary anaplastic astrocytoma in a child who received chemoradia-tion therapy for medulloblastoma after surgical treatment. Purpose. Demonstration of a rare occurrence of anaplastic astrocytoma after surgical and chemoradiation treatment of medulloblastoma.