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Indian Journal of Pathology &... Jun 2024Ependymomas exhibit heterogeneity across age, location, histology, molecular nature and survival suggestive of an epigenetic component in its pathogenesis. The CNS WHO...
BACKGROUND AND AIMS
Ependymomas exhibit heterogeneity across age, location, histology, molecular nature and survival suggestive of an epigenetic component in its pathogenesis. The CNS WHO classification (2021) classifies ependymomas based on DNA methylation profiles. Studies suggest that molecular sub-types remain stable throughout the course of disease. Immunohistochemical expression of L1CAM, has been identified as a surrogate marker for ZFTA/c11orf95-RELA fusion in supratentorial ependymomas. This study aims at realising its utility specially in resource-poor setups.
MATERIALS AND METHODS
Forty-three histopathologically-proven cases of ependymoma under treatment over the period of three years were selected. Histopathological examination followed by routine IHC staining for GFAP, S-100, EMA and Ki-67 in all cases and L1CAM in the supratentorial ependymomas was done. We have followed-up almost all cases during our study period and was correlated with the IHC expression patterns and clinico-pathological parameters, including survival.
RESULTS
In our study the commonest location for ependymomas was spine in adults and posterior fossa in pediatric age group. Majority cases belonged to CNS WHO Grade 2 both in adults and in the paediatric age group. Supratentorial location of ependymomas with positive immuno-reactivity for L1CAM and a higher Ki-67 labelling index were associated with poor survival.
CONCLUSION
Our study revealed that L1CAM is an effective surrogate marker for supratentorial ependymomas possibly carrying the ZFTA Fusion gene product. The L1CAM immuno-reactivity also corresponded with the survival data. However, larger population-based studies are required to validate these results further.
PubMed: 38904443
DOI: 10.4103/ijpm.ijpm_814_23 -
European Journal of Pharmaceutics and... Jun 2024Core-shell particles composed of polycaprolactone/polyvinyl alcohol (PCL/PVA) with pH sensitive properties were successfully fabricated by co-axial electrospraying in...
Core-shell particles composed of polycaprolactone/polyvinyl alcohol (PCL/PVA) with pH sensitive properties were successfully fabricated by co-axial electrospraying in which PVA and PCL formed the shell and core layers respectively. The core-shell structure was confirmed by FTIR, DSC and SEM analysis. No chemical interaction between PVA and PCL core-shell were observed in the FTIR analysis. The RAD001 loaded core-shell particles showed a sustained and pH dependent drug release and was assayed via our previously developed HPLC method. After indirect treatment of the PF-A cells with the core-shell particles for 24 h and 5 days a decrease in cell viability was observed. Additionally, a comparison was made with our previously developed nanoparticles containing 2 %PVA-14 %SOL®-0.6 % RAD001, for the cell viability study on ependymoma. Our findings show that optimised core-shell particles exerted a significant effect for the 24 h and 5 day treatment however further studies are required to ensure toxicity of the control core-shell particles with no drug is reduced. In comparison, the 2 %PVA-14 %SOL®-0.6 %RAD001 uniaxial electrosprayed nanoparticles also exerted a toxicity effect decreasing cell viability with no toxicity observed for the control nanoparticles as well. Such pH-sensitive core-shell particles, which can degrade effectively in either acidic or neutral condition, have great potential for application in the biomedical field.
PubMed: 38901620
DOI: 10.1016/j.ejpb.2024.114376 -
Neurologia Medico-chirurgica Jun 2024NF2-related schwannomatosis (NF2; previously termed neurofibromatosis type 2) is a tumor-prone disorder characterized by development of multiple schwannomas and...
NF2-related schwannomatosis (NF2; previously termed neurofibromatosis type 2) is a tumor-prone disorder characterized by development of multiple schwannomas and meningiomas. The diagnostic criteria of NF2 have been regularly revised. Clinical criteria for NF2 were first formulated at the National Institutes of Health Consensus Conference in 1987 and revised in 1990. Revised criteria were also proposed by the Manchester group in 1992 and by the National Neurofibromatosis Foundation (NNFF) in 1997. The 2011 Baser criteria improved the sensitivity of diagnostic criteria, particularly for patients without bilateral vestibular schwannomas. Revisions to the Manchester criteria were published in 2019, with replacement of "glioma" by "ependymoma," removal of "neurofibroma," addition of an age limit of 70 years for development of vestibular schwannomas, and introduction of molecular criteria, which led to the most widely used criteria. In 2022, the criteria were reviewed and updated by the international committee of NF experts. In addition to changes in diagnostic criteria, the committee recommended the use of "schwannomatosis" as an umbrella term for conditions that predispose to schwannomas. Each type of schwannomatosis was classified by the gene containing the disease-causing pathogenic variant. Molecular data from NF2 patients led to further clarification of the diagnostic criteria for NF2 mosaic phenotypes. Given all these changes, the diagnostic criteria of NF2 may be confusing. Herein, to help healthcare professionals who diagnose NF2 conditions in the clinical setting, we review the historical development of diagnostic criteria.
PubMed: 38897938
DOI: 10.2176/jns-nmc.2024-0067 -
Cancers May 2024(1) Background: Myxopapillary ependymoma (MPE) is a rare tumor of the spine, typically slow-growing and low-grade. Optimal management strategies remain unclear due to...
(1) Background: Myxopapillary ependymoma (MPE) is a rare tumor of the spine, typically slow-growing and low-grade. Optimal management strategies remain unclear due to limited evidence given the low incidence of the disease. (2) Methods: We analyzed data from 1197 patients with spinal MPE from the Surveillance, Epidemiology, and End Results (SEER) database (2000-2020). Patient demographics, treatment modalities, and survival outcomes were examined using statistical analyses. (3) Results: Most patients were White (89.9%) with a median age at diagnosis of 42 years. Surgical resection was performed in 95% of cases. The estimated 10-year overall survival was 91.4%. Younger age (hazard ratio (HR) = 1.09, < 0.001) and receipt of surgery (HR = 0.43, = 0.007) were associated with improved survival. Surprisingly, male sex was associated with worse survival (HR = 1.86, = 0.008) and a younger age at diagnosis compared to females. (4) Conclusions: This study, the largest of its kind, underscores the importance of surgical resection in managing spinal MPE. The unexpected association between male sex and worse survival warrants further investigation into potential sex-specific pathophysiological factors influencing prognosis. Despite limitations, our findings contribute valuable insights for guiding clinical management strategies for spinal MPE.
PubMed: 38893133
DOI: 10.3390/cancers16112013 -
Frontiers in Oncology 2024A 53-year-old male patient presented progressive numbness and weakness in the right limbs for a 2-year duration. Magnetic resonance imaging scans revealed an...
A 53-year-old male patient presented progressive numbness and weakness in the right limbs for a 2-year duration. Magnetic resonance imaging scans revealed an intramedullary lesion crossed over cervical and thoracic levels accompanied by syringomyelia at the proximal end of the lesion. The patient underwent subtotal resection of the neoplasm. The histological findings of the tumor were consistent with primary intramedullary malignant melanoma and not initial ependymoma after careful dermatologic and ophthalmologic re-examination. Primary melanoma of the spinal cord, particularly cervicothoracic localization with syringomyelia, is seldom reported in the literature. We report a case of this uncommon tumor and also discuss the clinical course, diagnosis, and treatment.
PubMed: 38863638
DOI: 10.3389/fonc.2024.1417268 -
The Journal of the Canadian... Apr 2024Lhermitte's sign is a nonspecific historical and exam finding that carries with it a differential diagnosis of cervical myelopathy, multiple sclerosis, intradural...
Spinal ependymoma presenting as subtle neurological findings in a VA chiropractic clinic: a case report in differential diagnosis and appropriate use of diagnostic imaging.
BACKGROUND
Lhermitte's sign is a nonspecific historical and exam finding that carries with it a differential diagnosis of cervical myelopathy, multiple sclerosis, intradural tumors, or other central nervous system pathology. Regardless of the suspected diagnosis, further diagnostic investigation is indicated to determine etiology of symptoms.
CASE PRESENTATION
In this case, a 67-year-old male Veteran presents to a Veterans Affairs (VA) outpatient chiropractic clinic with an insidious 6-month onset of neck pain with historical description of a positive Lhermitte's sign, a single episode of bladder incontinence, and mild changes in upper extremity manual dexterity. These subtle historical findings prompted referral for a brain and cervical spine MRI, revealing an ependymoma in the cervical spine. Urgent neurosurgical referral was made, and the patient underwent C3-C7 laminectomy, C3-T2 fusion, and tumor resection.
SUMMARY
This case represents an example of clinical reasoning in a VA chiropractic clinic when presented with subtle neurologic findings, and discusses the differential diagnoses and decision-making process to pursue imaging that resulted in appropriate neurosurgical management.
PubMed: 38840970
DOI: No ID Found -
SA Journal of Radiology 2024Leptomeningeal dissemination is a rare manifestation of pilocytic astrocytoma. It may occur with higher-grade tumours like medulloblastoma, ependymoma and high-grade...
UNLABELLED
Leptomeningeal dissemination is a rare manifestation of pilocytic astrocytoma. It may occur with higher-grade tumours like medulloblastoma, ependymoma and high-grade glioma, but is extremely rare with low-grade glioma. There has been a growing number of reported cases documenting leptomeningeal dissemination of pilocytic astrocytoma in the medical literature.
CONTRIBUTION
Description of a World Health Organization (WHO) Grade I suprasellar pilocytic astrocytoma with leptomeningeal dissemination in the brain and spinal cord which showed progression of the leptomeningeal nodules without tumour upgrading on long-term follow-up.
PubMed: 38840827
DOI: 10.4102/sajr.v28i1.2876 -
Neurology India Mar 2024
Endoscopic Endonasal Inter-dural Posterior Clinoidectomy and Pituitary Hemitranspostion for Surgical Resection of a Large Suprasellar Ependymoma Involving the Inter-peduncular Cistern.
Topics: Humans; Ependymoma; Neuroendoscopy; Pituitary Gland; Male; Neurosurgical Procedures; Magnetic Resonance Imaging; Adult; Female
PubMed: 38817169
DOI: 10.4103/neurol-india.Neurol-India-D-24-00115 -
Cureus Apr 2024Neuroepithelial tumors known as ependymomas can develop from cortical rests, the central canal of the spinal cord, or the ependymal cells of the cerebral ventricles....
Neuroepithelial tumors known as ependymomas can develop from cortical rests, the central canal of the spinal cord, or the ependymal cells of the cerebral ventricles. Ependymomas may arise anywhere along the neuraxis. Here, we present a 40-year-old male, a known case of grade II ependymomas, with a chief complaint of bilateral lower limb weakness and loss of sensation in the bilateral lower limb for 20 days. He started facing difficulties in performing activities such as walking, toileting activities, and squatting activities. The physiotherapy (PT) rehabilitation of the patient was tailored to achieve functional independence of the patient. The treatment session lasted for six weeks. Several outcome indicators were employed to evaluate our patient's progress toward functional recovery. Outcomes are measured using the Tone Grading Scale (TGS), the American Spinal Injury Association (ASIA) Impairment Scale, the World Health Organization Quality of Life (WHOQOL), manual muscle test, and the Barthel Index. Outcome measures were assessed on day one of treatment and the last day of the PT treatment. The patient's preliminary involvement in PT supported him to prevent serious complications like joint contractures and bed sores. Physical therapy is one of the most important parts of the rehabilitation practice for spinal cord injury (SCI) patients.
PubMed: 38784337
DOI: 10.7759/cureus.58809