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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 -
Neuro-oncology Jun 2024Neurocognition can be severely affected in pediatric brain tumor survivors. We analyzed the association of cognitive functioning with radiotherapy dose, postoperative...
BACKGROUND
Neurocognition can be severely affected in pediatric brain tumor survivors. We analyzed the association of cognitive functioning with radiotherapy dose, postoperative cerebellar mutism syndrome (pCMS), hydrocephalus, intraventricular methotrexate (MTX) application, tumor localization and biology in pediatric survivors of a posterior fossa tumor.
METHODS
Subdomain-specific neurocognitive outcome data from 279 relapse-free survivors of the HIT-2000 trial (241 medulloblastoma and 38 infratentorial ependymoma) using the Neuropsychological Basic Diagnostic (NBD) tool based on Cattell-Horn-Carroll's model for intelligence were analyzed.
RESULTS
Cognitive performance 5.14 years (mean; range=1.52-13.02) after diagnosis was significantly below normal for all subtests. Processing speed and psychomotor abilities were most affected. Influencing factors were domain-specific: CSI-dose had strong impact on most subtests. pCMS was associated with psychomotor abilities (β=-0.25 to -0.16) and processing speed (β=-0.32). Postoperative hydrocephalus correlated with crystallized intelligence (β=-0.20) and short-term memory (β=-0.15), age with crystallized intelligence (β=0.15) and psychomotor abilities (β=-0.16 and β=-0.17). Scores for fluid intelligence (β=-0.23), short-term memory (β=-0.17) and visual processing (β=-0.25) declined, and scores for selective attention improved (β=0.29) with time after diagnosis.
CONCLUSION
Dose of CSI was strongly associated with neurocognitive outcome. Low psychomotor abilities and processing speed both in patients treated with and without CSI suggest a strong contribution of the tumor and its surgery on these functions. Future research therefore should analyze strategies to both reduce CSI-dose and toxicity caused by other treatment modalities.
PubMed: 38835160
DOI: 10.1093/neuonc/noae092 -
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; Magnetic Resonance Imaging; Neuroendoscopy; Neurosurgical Procedures; Pituitary Gland
PubMed: 38817169
DOI: 10.4103/neurol-india.Neurol-India-D-24-00115 -
World Neurosurgery May 2024Myxopapillary ependymoma are well circumscribed tumours arising mainly from conus medullaris (CM) and filum terminale (FT), typically presenting at median age of 39...
Myxopapillary ependymoma are well circumscribed tumours arising mainly from conus medullaris (CM) and filum terminale (FT), typically presenting at median age of 39 years. Owing to its aggressive clinical behaviour including cerebrospinal fluid dissemination and local recurrence, it is classified as grade 2 in World Health Organisation Central Nervous System 5 Classification. Gross total resection without capsular violation is critical, as subtotal resection is associated with local recurrence. The FT comprises intradural filum terminale (iFT) and extradural filum terminalecomponents with iFT extending from the inferior tip of the CM to coccyx. The iFT-CM junction is a transitional zone; with neural tissue being incrementally replaced by fibrous tissue of filum, gradually converging to a pure nonneural FT. Achieving gross total resection is challenging for intramedullary FT myxopapillary ependymoma in proximity to conus, necessitating neuromonitoring to preserve vital CM functions. We present a case of 33-year-old male with 6 months of nocturnal back pain and bilateral lower limb without neurological deficits. Preoperative MRI revealed a T2 hyperintense, heterogeneously contrast enhancing intradural extramedullary mass at L1 vertebral level.
PubMed: 38796147
DOI: 10.1016/j.wneu.2024.05.111 -
Neurosurgery May 2024Ependymoma is commonly classified as World Health Organization grade 2 with the anaplastic variant categorized as grade 3. Incomplete resection or anaplastic features...
BACKGROUND AND OBJECTIVES
Ependymoma is commonly classified as World Health Organization grade 2 with the anaplastic variant categorized as grade 3. Incomplete resection or anaplastic features can result in unfavorable outcomes. Stereotactic radiosurgery (SRS) provides a minimally invasive approach for recurrent ependymomas. Our study investigates the efficacy and safety of SRS for grade 2 and 3 ependymomas in pediatric and adult populations.
METHODS
We conducted a retrospective analysis on 34 patients with 75 ependymomas after CyberKnife SRS between 1998 and 2023. Fourteen were pediatric (3-18 years), and 20 were adult (19-75 years) patients. The median age was 21 years, and the median tumor volume was 0.64 cc. The median single-fraction equivalent dose was 16.6 Gy, with SRS administered at 77% of the median isodose line.
RESULTS
After a median follow-up of 42.7 months (range: 3.8-438.3), 22.7% of ependymomas progressed. The 5-year local tumor control rate was 78.1%, varying between 59.6% and 90.2% for children and adults, with grade 2 at 85.9% compared with 58.5% for grade 3 tumors. The 5-year overall survival rate was 73.6%, notably higher in adults (94.7%) than in children (41%), and 100% for grade 2 but decreased to 35.9% for grade 3 patients. The 5-year progression-free survival rate was 68.5%, with 78.3% and 49.2% for adults and children, respectively, and a favorable 88.8% for grade 2, contrasting with 32.6% for grade 3 patients. Symptom improvement was observed in 85.3% of patients. Adverse radiation effects occurred in 21.4% of pediatric patients.
CONCLUSION
Our study supports SRS as a viable modality for pediatric and adult patients with grade 2 and 3 ependymomas. Despite lower local tumor control in pediatric and grade 3 cases, integrating SRS holds promise for improved outcomes. Emphasizing careful patient selection, personalized treatment planning, and long-term follow-up is crucial for optimal neurosurgical outcomes.
PubMed: 38785440
DOI: 10.1227/neu.0000000000002979 -
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 -
AJNR. American Journal of Neuroradiology May 2024The aim of this study was to determine the diagnostic value of fractional plasma volume derived from dynamic contrast-enhanced perfusion MR imaging versus ADC, obtained...
BACKGROUND AND PURPOSE
The aim of this study was to determine the diagnostic value of fractional plasma volume derived from dynamic contrast-enhanced perfusion MR imaging versus ADC, obtained from DWI in differentiating between grade 2 (low-grade) and grade 3 (high-grade) intracranial ependymomas.
MATERIALS AND METHODS
A hospital database was created for the period from January 2013 through June 2022, including patients with histologically-proved ependymoma diagnosis with available dynamic contrast-enhanced MR imaging. Both dynamic contrast-enhanced perfusion and DWI were performed on each patient using 1.5T and 3T scanners. Fractional plasma volume maps and ADC maps were calculated. ROIs were defined by a senior neuroradiologist manually by including the enhancing tumor on every section and conforming a VOI to obtain the maximum value of fractional plasma volume (Vp) and the minimum value of ADC (ADC). A Mann-Whitney test at a significance level of corrected = .01 was used to evaluate the differences. Additionally, receiver operating characteristic curve analysis was applied to assess the sensitivity and specificity of Vp and ADC values.
RESULTS
A total of 20 patients with ependymomas (10 grade 2 tumors and 10 grade 3 tumors) were included. Vp values for grade 3 ependymomas were significantly higher ( < .002) than those for grade 2. ADC values were overall lower in high-grade lesions. However, no statistically significant differences were found ( = .12114).
CONCLUSIONS
As a dynamic contrast-enhanced perfusion MR imaging metric, fractional plasma volume can be used as an indicator to differentiate grade 2 and grade 3 ependymomas. Dynamic contrast-enhanced perfusion MR imaging plays an important role with high diagnostic value in differentiating low- and high-grade ependymoma.
PubMed: 38782589
DOI: 10.3174/ajnr.A8226 -
Pediatric Neurology Jul 2024
Review
Topics: Humans; Ependymoma; Neoplasms, Neuroepithelial; Brain Neoplasms; Child
PubMed: 38781722
DOI: 10.1016/j.pediatrneurol.2024.04.022 -
Pediatric Emergency Care May 2024Due to the varied symptomatology and inconsistent features on neurologic exam, central nervous system (CNS) tumors are difficult to diagnosis in a timely manner.
Clinical Characteristics and Delayed Diagnosis of Pediatric Patients Presenting to the Emergency Department With a Newly Diagnosed Central Nervous System Tumor: A Single Institutional Experience.
BACKGROUND
Due to the varied symptomatology and inconsistent features on neurologic exam, central nervous system (CNS) tumors are difficult to diagnosis in a timely manner.
OBJECTIVE
To determine the clinical, neurological, and neuroimaging features of newly diagnosed CNS tumors presenting to the emergency department (ED).
METHODS
We evaluated a retrospective cohort of 121 consecutive patients presenting to a tertiary care pediatric ED over 7 consecutive years with newly diagnosed CNS tumors. Clinical symptomatology, neurologic findings reported by emergency room and neurology physicians, neuroimaging features, and time to diagnosis were analyzed.
RESULTS
A total of 116 (48 female, median age 8.0 years (interquartile range, 4.4-12.6), 52% Hispanic) presented to the ED (64% self-referred) diagnosed with a brain tumor (54% posterior fossa, 24% embryonal, 24% low-grade glioma, 16% high-grade glioma) resulting in hospital admission in 92% of cases. Five were diagnosed with extradural spinal, clivus, or orbital apex tumors. Symptomatology or duration did not differ when stratified by demographics, location, or histologic subtype. Moderate degree of concordance was observed among neurologic examinations performed by ED physicians and neurologists. Delayed diagnosis (median delay = 3.5 [1-7] months) was seen in 14% of patients, 13 with primary brain tumors (11 hemispheric, 2 brain stem). Six children with delayed diagnosis of low-grade glial tumors had a nonfocal neurologic examination in comparison to 5 patients with abnormal examinations observed with primary spinal or extradural CNS tumors. Four patients with posterior fossa tumors (3 medulloblastoma, 1 ependymoma) had normal/near normal neurologic examination at presentation despite posterior fossa symptomatology related to increased intracranial pressure.
CONCLUSIONS
Our series highlights the complexity of symptomology and neurologic findings in children presenting to the ED with newly diagnosed CNS tumors who may have a normal neurologic examination. Standardization of symptom assessment and focused neurologic examinations may lead to earlier neuroimaging and prevent delayed diagnosis.
PubMed: 38776448
DOI: 10.1097/PEC.0000000000003227