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Advanced Biomedical Research 2023Given that spinal cord tumors cause damage to the central nervous system, the involvement of body organs, patients' reduced quality of life, and the follow-up of...
BACKGROUND
Given that spinal cord tumors cause damage to the central nervous system, the involvement of body organs, patients' reduced quality of life, and the follow-up of patients should be performed with more sensitivity. Therefore, the present study aimed to determine the demographic characteristics and treatment follow-up of patients with spinal cord tumors.
MATERIALS AND METHODS
The present descriptive-analytical and cross-sectional study investigated all patients with spinal cord tumors in 2022 who visited the Kashani Hospital Isfahan. A total of 31 patients were included in the study using the census sampling method. The data were collected by a checklist and three pain scales, namely the Dennis pain scale, the Frankel Grade classification, and Nurick's grading scales.
RESULTS
The present cross-sectional study examined 31 patients with spinal cord tumors who visited the Kashani Hospital in Isfahan. Most spinal cord tumors were ependymoma with nine cases (29%) and neurofibroma with six cases (19.35%). The tumor site was thoracic in 10 cases (33.33%). In the follow-up phase, 31 patients (53.4%) returned to their normal life with full recovery and 10 (17.2%) died. Three (75%) out of four cases of tumor recurrence had ependymoma tumors and one (25%) had astrocytoma. There was a significant difference between the patients' pre- and post-operative pain severity and functional status as the post-operative scores decreased on all three scales.
CONCLUSION
The results may contribute to improve the pre-operative planning, optimization, and monitoring of the patient's recovery after the operation.
PubMed: 38073727
DOI: 10.4103/abr.abr_168_23 -
Molecular Therapy. Methods & Clinical... Dec 2023We report partial response (PR) to novel therapy with selumetinib in a patient with neurofibromatosis type 2 (NF2). A 25-year-old male presented with bilateral...
We report partial response (PR) to novel therapy with selumetinib in a patient with neurofibromatosis type 2 (NF2). A 25-year-old male presented with bilateral vestibular schwannomas, spinal cord intramedullary ependymomas, cranial and spinal meningiomas, spinal nerve root mixed schwannoma-neurofibromas, and peripheral nerve sheath tumors. He tested negative for germline , SWItch/sucrose non-fermentable-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (, and leucine zipper-like transcription regulator 1 ( mutations. Molecular analysis of a resected cervical spine schwannoma-neurofibroma demonstrated an isolated somatic mutation. Due to progression of all tumors, he was treated medically with both everolimus (10 mg/day) and selumetinib (25 mg/kg twice a day), but he rapidly transitioned to selumetinib monotherapy due to everolimus toxicity. 3 months of treatment resulted in PR in one spinal ependymoma and stable disease in other tumors. This PR was quantified by the differences in units of intensity in pre- and post-treatment magnetic resonance image. To the best of our knowledge, this is the first reported case for using selumetinib in NF2-associated tumors or ependymomas.
PubMed: 38058737
DOI: 10.1016/j.omtm.2023.101156 -
Frontiers in Molecular Biosciences 2023Pediatric brain tumours (PBT) are one of the most common malignancies during childhood, with variable severity according to the location and histological type. Certain...
Pediatric brain tumours (PBT) are one of the most common malignancies during childhood, with variable severity according to the location and histological type. Certain types of gliomas, such a glioblastoma and diffuse intrinsic pontine glioma (DIPG), have a much higher mortality than ependymoma and medulloblastoma. Early detection of PBT is essential for diagnosis and therapeutic interventions. Liquid biopsies have been demonstrated using cerebrospinal fluid (CSF), mostly restricted to cell free DNA, which display limitations of quantity and integrity. In this pilot study, we sought to demonstrate the detectability and robustness of cell free histones in the CSF. We collected CSF samples from a pilot cohort of 8 children with brain tumours including DIPG, medulloblastoma, glioblastoma, ependymoma and others. As controls, we collected CSF samples from nine children with unrelated blood malignancies and without brain tumours. We applied a multichannel flow imaging approach on ImageStream(X) to image indiviual histone or histone complexes on different channels. Single histones (H2A, macroH2A1.1, macroH2A1.2 H2B, H3, H4 and histone H3 bearing the H3K27M mutation), and histone complexes are specifically detectable in the CSF of PBT patients. H2A and its variants macroH2A1.1/macroH2A1/2 displayed the strongest signal and abundance, together with disease associated H3K27M. In contrast, mostly H4 is detectable in the CSF of pediatric patients with blood malignancies. In conclusion, free histones and histone complexes are detectable with a strong signal in the CSF of children affected by brain tumours, using ImageStream(X) technology and may provide additive diagnostic and predictive information.
PubMed: 38028540
DOI: 10.3389/fmolb.2023.1254699 -
Journal of Applied Clinical Medical... Jan 2024To study the dosimetric impact of incorporating variable relative biological effectiveness (RBE) of protons in optimizing intensity-modulated proton therapy (IMPT)...
PURPOSE
To study the dosimetric impact of incorporating variable relative biological effectiveness (RBE) of protons in optimizing intensity-modulated proton therapy (IMPT) treatment plans and to compare it with conventional constant RBE optimization and linear energy transfer (LET)-based optimization.
METHODS
This study included 10 pediatric ependymoma patients with challenging anatomical features for treatment planning. Four plans were generated for each patient according to different optimization strategies: (1) constant RBE optimization (ConstRBEopt) considering standard-of-care dose requirements; (2) LET optimization (LETopt) using a composite cost function simultaneously optimizing dose-averaged LET (LET ) and dose; (3) variable RBE optimization (VarRBEopt) using a recent phenomenological RBE model developed by McNamara et al.; and (4) hybrid RBE optimization (hRBEopt) assuming constant RBE for the target and variable RBE for organs at risk. By normalizing each plan to obtain the same target coverage in either constant or variable RBE, we compared dose, LET , LET-weighted dose, and equivalent uniform dose between the different optimization approaches.
RESULTS
We found that the LETopt plans consistently achieved increased LET in tumor targets and similar or decreased LET in critical organs compared to other plans. On average, the VarRBEopt plans achieved lower mean and maximum doses with both constant and variable RBE in the brainstem and spinal cord for all 10 patients. To compensate for the underdosing of targets with 1.1 RBE for the VarRBEopt plans, the hRBEopt plans achieved higher physical dose in targets and reduced mean and especially maximum variable RBE doses compared to the ConstRBEopt and LETopt plans.
CONCLUSION
We demonstrated the feasibility of directly incorporating variable RBE models in IMPT optimization. A hybrid RBE optimization strategy showed potential for clinical implementation by maintaining all current dose limits and reducing the incidence of high RBE in critical normal tissues in ependymoma patients.
Topics: Child; Humans; Proton Therapy; Radiotherapy Dosage; Relative Biological Effectiveness; Linear Energy Transfer; Ependymoma; Radiotherapy Planning, Computer-Assisted; Organs at Risk
PubMed: 37985962
DOI: 10.1002/acm2.14207 -
The Primary Care Companion For CNS... Nov 2023
Topics: Humans; Glioma, Subependymal; Capgras Syndrome; Craniotomy
PubMed: 37976231
DOI: 10.4088/PCC.23cr03524 -
International Journal of Clinical and... 2023An unusual, small cell-predominant, high-grade glioneuronal tumor in the occipital lobe of a 49-year-old man that co-existed with a low-grade tumor is reported. The...
An unusual, small cell-predominant, high-grade glioneuronal tumor in the occipital lobe of a 49-year-old man that co-existed with a low-grade tumor is reported. The tumor consisted of two distinct components: the major component was a dense proliferation of primitive small cells showing bidirectional neuronal and glial differentiation; and the minor component consisted of a proliferation of well-differentiated astrocytes intermingled with mature neuronal cells. In the former component, perivascular pseudorosette-like or pseudopapillary growth reminiscent of ependymoma or papillary glioneuronal tumor (PGNT), respectively, was prominent, and hypertrophic astrocytic cells were located just outside the central blood vessels. Small cells were immunoreactive for Olig2, synaptophysin, and, less frequently, for glial fibrillary acidic protein. The low-grade component included Rosenthal fibers, hemosiderin deposition, and perivascular lymphocytic infiltration, thus closely resembling ganglioglioma. Cytogenetic studies did not demonstrate any mutations or rearrangements of the genes or promoter. The tumor recurred and spread along the ventricular surface three years after total removal. The small cell-predominant, high-grade component was considered to have evolved from the ganglioglioma-like, low-grade component. The histopathologic resemblance of the high-grade component to PGNT was a special feature.
PubMed: 37970335
DOI: No ID Found -
Brain Tumor Research and Treatment Oct 2023Ependymoma is a rare adult tumor that originates from ependymal cells of the central nervous system, primarily occurring in the cerebral ventricles or the central canal...
Ependymoma is a rare adult tumor that originates from ependymal cells of the central nervous system, primarily occurring in the cerebral ventricles or the central canal of the spinal cord. In this paper, we report a case of extensive leptomeningeal seeding of ependymoma of a 39-year-old male patient, in whom the tumor was found incidentally after head trauma. The MRI exhibited diffuse leptomeningeal infiltrative lesions along with bilateral multiple cerebral sulci, basal cisterns, cerebellopontine angle, cerebellar folia. It also showed multinodular enhancing T1 low T2 high signal intensity lesions along the whole spinal cord. After the tumor biopsy at right temporal lesion, pathologic diagnosis was classic ependymoma (WHO grade 2). The patient has undergone radiation therapy and chemotherapy, and is currently maintaining a stable condition two years after surgery. This report suggests that when considering the differential diagnosis of extensive lesions both in the intracranial and intraspinal space, ependymoma should also be considered.
PubMed: 37953452
DOI: 10.14791/btrt.2023.0029 -
The American Journal of Surgical... Jan 2024The 2021 World Health Organization classification of tumors of the central nervous system emphasizes the significance of molecular parameters for an integrated...
The 2021 World Health Organization classification of tumors of the central nervous system emphasizes the significance of molecular parameters for an integrated diagnosis. Homozygous deletion of cyclin-dependent kinase inhibitor 2a (CDKN2A) has been associated with an adverse prognosis in IDH -mutant gliomas, supratentorial ependymomas, meningiomas, and MPNST. In this study, we examined the value of p16 protein immunohistochemistry as a rapid and cost-effective screening tool for a homozygous CDKN2A deletion. Genetic analyses for CDKN2A in 30 pleomorphic xanthoastrocytomas, 32 IDH -wild-type high-grade gliomas, 40 supratentorial ependymomas with ZFTA-RELA gene fusion, 21 IDH-mutant astrocytomas, and 24 meningiomas were performed mainly by a molecular inversion probe assay, a high-resolution, quantitative technology for the assessment of chromosomal copy number alterations. Immunohistochemistry for p16 proved to have a high positive predictive value (range 90% to 100%) and an overall low negative predictive value (range 22% to 93%) for a homozygous CDKN2A deletion. In a setting where molecular testing is limited for cost and time reasons, p16 immunohistochemistry serves as a useful and rapid screening tool for identifying cases that should be subjected to further molecular testing for CDKN2A deletions.
Topics: Humans; Immunohistochemistry; Meningioma; Homozygote; Sequence Deletion; Cyclin-Dependent Kinase Inhibitor p16; Glioma; Meningeal Neoplasms; Ependymoma; Gene Deletion
PubMed: 37947008
DOI: 10.1097/PAS.0000000000002148 -
Frontiers in Neurology 2023Brain tumors, especially gliomas, are known for high lethality. It is currently understood that the correlations of tumors with coagulation and inflammation have been...
BACKGROUND
Brain tumors, especially gliomas, are known for high lethality. It is currently understood that the correlations of tumors with coagulation and inflammation have been gradually revealed.
OBJECTIVE
This study aimed to explore the potential value of several reported peripheral blood parameters as comprehensively as possible, with preoperative diagnosis and identification of brain tumors (focus on gliomas).
METHODS
Patients with central nervous system tumors (craniopharyngioma, ependymoma, spinal meningioma, acoustic neuroma, brain metastases, meningioma, and glioma) or primary trigeminal neuralgia admitted to our hospital were retrospectively analyzed. The results of the routine coagulation factor test, serum albumin test, and blood cell test in peripheral blood were recorded for each group of patients on admission. Neutrophil-lymphocyte ratio (NLR), derived NLR (dNLR), platelet-lymphocyte ratio (PLR), lymphocyte-monocyte ratio (LMR), prognostic nutritional index (PNI), the systemic immune-inflammation index (SII), pan-immune-inflammation value (PIV), and their pairings were calculated. Their ability to identify brain tumors and their correlation with glioma grade were analyzed.
RESULTS
A total of 698 patients were included in this retrospective case-control study. Glioma patients had higher NLR, SII, and PIV but lower LMR. The NLR in the brain metastasis group was lower than that in the control, meningioma, and acoustic neuroma groups, but the SII and PIV were higher than those in the ependymoma group. Fibrinogen, white blood cell count, neutrophil count, NLR, SII, and PIV in the GBM group were higher than those in the control group. In all comparisons, NLR and NLR + dNLR showed the greatest accuracy, with areas under the curve (AUCs) of 0.7490 (0.6482-0.8498) and 0.7481 (0.6457-0.8505), respectively. PIV, dNLR + PIV, and LMR + PIV ranked second, with AUCs of 0.7200 (0.6551-0.7849), 0.7200 (0.6526-0.7874), 0.7204 (0.6530-0.7878) and 0.7206 (0.6536-0.7875), respectively.
CONCLUSION
NLR, PIV, and their combinations show high sensitivity and specificity in the diagnosis of brain tumors, especially gliomas. Overall, our results provide evidence for these convenient and reliable peripheral blood markers.
PubMed: 37936915
DOI: 10.3389/fneur.2023.1297835 -
Neurology India 2023Lipogenic differentiation in ependymoma is an infrequent occurrence with very few reported cases. The grading was done solely based on the histomorphology and molecular...
Lipogenic differentiation in ependymoma is an infrequent occurrence with very few reported cases. The grading was done solely based on the histomorphology and molecular subtyping was not described in such ependymomas. New molecular classification divided ependymomas in nine different subgroups, of which supratentorial location tumor usually exhibits C11orf95-RELA, YAP1-MAMLD1, and YAP1-FAM118B fusion proteins. A 46-year-old female presented with headache and right-sided parapresis. Radilogy revealed a large intraxial left parietooccipital mass lesion, which histologically and immuohistochemically confirmed as anaplastic ependymoma with extensive lipogenic changes. The ependymal origin of the tumor was corroborated by the immunohistochemistry and ultrastructural studies. Molecular studies for C11orf95-RELA, YAP1-MAMLD1, and YAP1-FAM118B fusion proteins were negative. This is the first documentation of fusion negative supratentorial anaplastic ependymoma with lipogenic differentiation. This novel finding needs further reinforcement by similar studies to identify its impact on the disease outcome.
Topics: Female; Humans; Middle Aged; Transcription Factor RelA; Supratentorial Neoplasms; Ependymoma; Immunohistochemistry; DNA-Binding Proteins; Nuclear Proteins; Transcription Factors; Proteins
PubMed: 37929446
DOI: 10.4103/0028-3886.388099