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Journal of Neuro-oncology May 2024The purpose of this prospective pilot study was to evaluate the feasibility and effects of cognitive-motor intervention on the cognitive and motor abilities of pediatric...
The purpose of this prospective pilot study was to evaluate the feasibility and effects of cognitive-motor intervention on the cognitive and motor abilities of pediatric survivors of posterior fossa tumors. The study involved patients aged 7 to 18 years with cognitive deficits who had completed primary treatment for posterior fossa tumors. 25 participants (M=11.3 ± 2.93, 64% male; 17 medulloblastoma, 1 ependymoma, 1 desmoplastic medulloblastoma, 6 piloid astrocytoma; 22 in remission (M =45), 3 in stabilization (M=49)) were recruited from the Research Institute for Brain Development and Peak Performance. The intervention consisted of two phases with a 3-month break for home training, and a total duration of 6 months. Each phase lasted 7 weeks and included two assessment procedures (pre- and post-intervention) and 10 training sessions over a period of 5 weeks (two 3-hour sessions per week). At baseline and pre- and post-intervention, all participants underwent a battery of cognitive and motor tests. Each training session included gross motor training (GMT), graphomotor training (GT), and cognitive-motor training (CMT). Statistical analysis was performed using the Friedman test for repeated measures and post-hoc Durbin-Conover test. The results indicated significant improvements in visuospatial working memory, visual attention, eye-hand coordination, semantic verbal fluency, auditory-motor synchronization, reaction time, and a decrease in the rate of ataxia. These improvements remained stable even in the absence of direct intervention. The findings demonstrate positive effects and feasibility of the intervention and suggest the need for further research in this area including randomized controlled feasibility studies with a larger sample.
Topics: Humans; Male; Pilot Projects; Child; Female; Infratentorial Neoplasms; Adolescent; Cancer Survivors; Prospective Studies; Feasibility Studies
PubMed: 38489149
DOI: 10.1007/s11060-024-04636-z -
Frontiers in Neurology 2024Ependymomas mostly locate in the infratentorial region and often occur in children. Anaplastic ependymomas account for 45-47% of supratentorial and 15-17% of...
BACKGROUND
Ependymomas mostly locate in the infratentorial region and often occur in children. Anaplastic ependymomas account for 45-47% of supratentorial and 15-17% of infratentorial ependymomas, also known as malignant ependymomas. Adult supratentorial extraventricular anaplastic ependymoma (SEAE) is rare in clinical practice, and only a few cases have been reported so far, and there is no clinical study with large sample size. We report a case of adult supratentorial extraventricular anaplastic ependymoma in the occipital lobe with cerebrospinal fluid dissemination metastases.
CASE DESCRIPTION
A 58-year-old female patient presented with unexplained pain in multiple parts of the body for the past half a year, mainly manifested as pain in the head, abdomen and chest. On August, 2022, Head MRI of the patient showed abnormal signal shadow in the left occipital lobe, which was considered a malignant lesion. The patient underwent tumor resection under general anesthesia on September 3, 2022. Postoperative pathological examination showed anaplastic ependymoma. The postoperative follow-up head MRI showed multiple cerebrospinal fluid dissemination metastases in the brain.
CONCLUSION
Adult SEAE is a rare tumor with high malignancy and have a tendency to disseminate into the CSF, resulting in drop metastases. Immunohistochemistry is very important for the diagnosis of SEAE. It is recommended to administer adjuvant chemotherapy or radiation therapy appropriately after surgery, based on the tumor being completely resected as much as possible.
PubMed: 38481945
DOI: 10.3389/fneur.2024.1351674 -
Chinese Medical Journal Mar 2024
PubMed: 38479992
DOI: 10.1097/CM9.0000000000003040 -
World Neurosurgery May 2024Ependymoma is a central nervous system (CNS) tumor that arises from the ependymal cells of the brain's ventricles and spinal cord. The histopathology of ependymomas is...
BACKGROUND
Ependymoma is a central nervous system (CNS) tumor that arises from the ependymal cells of the brain's ventricles and spinal cord. The histopathology of ependymomas is indistinguishable regardless of the site of origin, and the prognosis varies. Recent studies have revealed that the development site and prognosis reflect the genetic background. In this study, we used genome-wide DNA methylation array analysis to investigate the epigenetic background of ependymomas from different locations treated at our hospital.
METHODS
Four cases of posterior fossa ependymomas and 11 cases of spinal ependymomas were analyzed.
RESULTS
DNA methylation profiling using the DKFZ methylation classifier showed that the methylation diagnoses of the 2 cases differed from the histopathological diagnoses, and 2 cases could not be classified. Tumor that spread from the brain to the spinal cord was molecularly distinguishable from other primary spinal tumors.
CONCLUSIONS
Although adding DNA methylation classification to conventional diagnostic methods may be helpful, the diagnosis in some cases remains undetermined. This may affect decision-making regarding treatment strategies and follow-up. Further investigations are required to improve the diagnostic accuracy of these tumors.
Topics: Humans; Ependymoma; DNA Methylation; Female; Male; Adult; Middle Aged; Child; Adolescent; Spinal Cord Neoplasms; Young Adult; Child, Preschool; Infratentorial Neoplasms; Aged
PubMed: 38479644
DOI: 10.1016/j.wneu.2024.03.013 -
Journal of Neurosciences in Rural... 2024Tanycytic ependymomas mostly occur in the spinal cord and it is the rarest histological subtype of ependymoma. A 29-year-old male was referred from the infertility...
Tanycytic ependymomas mostly occur in the spinal cord and it is the rarest histological subtype of ependymoma. A 29-year-old male was referred from the infertility clinic after serum prolactin levels were found to be elevated. Magnetic resonance imaging (MRI) brain showed an irregular necrotic lesion in the periventricular region of the left parietal lobe which had an intraventricular component and associated perilesional edema. In addition, a sellar mass with suprasellar extension was also found on the MRI. He was started on cabergoline therapy for macroprolactinoma and underwent a left parietal craniotomy, and microsurgical excision of the tumor using intraoperative neurosonographic guidance. Histologically, the tumor showed spindle cytologic features and poorly developed inconspicuous pseudorosettes, with areas of rounded nuclear profiles and perinuclear cytoplasmic clearing. Tumor cells were positive for vimentin, glial fibrillary acidic protein and S100, and negative for epithelial membrane antigen. Ki67 was <7%. He was diagnosed with tanycytic ependymoma and a coexistent prolactinoma. He received 10 cycles of image-guided radiotherapy. Post-operative imaging showed minimal residual tumor the size of which remained stable at 1-year follow-up scan. The pituitary macroadenoma regressed with cabergoline therapy and he clinically improved. This presentation of synchronous macroprolactinoma and tanycytic ependymoma has not been reported in the literature previously. An exhaustive literature review showed only 18 previously reported cases of supratentorial tanycytic ependymoma.
PubMed: 38476418
DOI: 10.25259/JNRP_217_2023 -
Oncology Mar 2024The study explored the failure pattern and clinical outcomes in patients with ependymoma undergoing radiotherapy.
INTRODUCTION
The study explored the failure pattern and clinical outcomes in patients with ependymoma undergoing radiotherapy.
METHODS
Between January 2004 and June 2022, we included 32 patients with ependymoma who underwent radiotherapy as part of the multimodality treatment at our institution. Of these, 27 (84.4%) underwent adjuvant radiotherapy, four received radiotherapy after local recurrence, and one received definitive CyberKnife radiotherapy (21 Gy in three fractions). The median prescribed dose was 54 Gy in patients who received conventional radiotherapy. We analyzed the local progression-free survival (LPFS), distant metastasis-free survival (DMFS), progression-free survival (PFS), overall survival (OS), and potential prognostic factors.
RESULTS
The median age was 29.8 years. Approximately 28.1% were pediatric patients. Fifteen tumors (46.9%) were World Health Organization (WHO) grade II, 10 (31.3%) were WHO grade III, and seven (22.8%) were WHO grade I. Among them, 15 patients (46.9%) had posterior fossa tumors, 10 (31.3%) had supratentorial tumors, and seven (22.8%) had spinal tumors. Of the 31 patients who underwent upfront surgical resection, 19 (61.3%) underwent gross total resection or near total resection. Seventeen of 19 patients with first failures (89.5%) had isolated local recurrences. Of the 19 patients with disease progression, 11 (57.9%) were disease-free or had stable disease after salvage therapy, and five (26.3%) had disease-related mortality. Most of the first local recurrences after radiotherapy occurred in the infield (13 of 16, 81.3%). The 5-year LPFS, DMFS, PFS, and OS rates were 48.5%, 89.6%, 45.1%, and 88.4%, respectively, at a median follow-up of 6.25 years. Subtotal resection was associated with poorer LPFS and PFS in patients with intracranial ependymoma (hazard ratio = 3.69, p = 0.018 for LPFS; hazard ratio = 3.20, p = 0.029 for PFS).
CONCLUSION
Incorporating radiotherapy into multimodal treatment has led to favorable outcomes in patients with ependymoma, and the extent of resection is a prognostic factor for the local control of intracranial ependymoma.
PubMed: 38471461
DOI: 10.1159/000538321 -
Journal of Medical Case Reports Mar 2024Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor...
BACKGROUND
Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management.
CASE PRESENTATION
We report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient's sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3-L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted.
CONCLUSIONS
Intradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.
Topics: Humans; Male; Middle Aged; Prognosis; Sarcoma, Ewing; Spinal Cord Neoplasms; Spinal Neoplasms
PubMed: 38459600
DOI: 10.1186/s13256-024-04384-8 -
Journal of Pediatric Hematology/oncology Apr 2024Surveillance magnetic resonance imaging (MRI) is routinely used to detect recurrence in pediatric central nervous system (CNS) tumors. The frequency of neuroimaging...
Surveillance magnetic resonance imaging (MRI) is routinely used to detect recurrence in pediatric central nervous system (CNS) tumors. The frequency of neuroimaging surveillance varies without a standardized approach. A single-institutional retrospective cohort study evaluated the frequency of recurrences. This study included 476 patients with the majority diagnosed with low-grade glioma (LGG) (n=138, 29%), high-grade glioma (HGG) (n=77, 16%), ependymoma (n=70, 15%), or medulloblastoma (n=61, 13%). LGG, HGG, and ependymoma patients more commonly had multiply recurrent disease ( P =0.08), with ependymoma patients demonstrating ≥2 relapses in 47% of cases. Recurrent disease was identified by imaging more often than clinical symptoms (65% vs. 32%; P =<0.01). Patients diagnosed with meningioma demonstrated the longest mean time to first relapse (74.7 mo) whereas those with atypical teratoid rhabdoid tumor and choroid plexus carcinoma tended to have the shortest time to relapse (8.9 and 9 mo, respectively). Overall, 22 patients sustained first relapse >10 years from initial diagnosis. With a higher tendency toward detection of tumor recurrence/progression on MRI surveillance in comparison to clinical progression, surveillance imaging is necessary in routine follow up of pediatric CNS tumor survivors. With some relapses >10 years from initial diagnosis, imaging beyond this time point may be useful in particular tumor types. While the study is limited in outcome analysis, earlier detection of recurrence would lead to earlier initiation of treatment and implementation of salvage treatment regimens which can impact survival and quality of life.
Topics: Child; Humans; Retrospective Studies; Quality of Life; Neoplasm Recurrence, Local; Brain Neoplasms; Glioma; Central Nervous System Neoplasms; Ependymoma; Magnetic Resonance Imaging; Cerebellar Neoplasms; Recurrence
PubMed: 38447113
DOI: 10.1097/MPH.0000000000002850 -
Journal of Neuro-oncology May 2024Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion...
PURPOSE
Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion extent of resection (EOR) has been studied, existing analyses are limited by small samples and archaic data resulting in an inhomogeneous population. We investigated the relationship between EOR and survival outcomes, chiefly overall survival (OS) and progression-free survival (PFS), in a large contemporary cohort of spinal ependymoma patients.
METHODS
Adult patients diagnosed with a spinal ependymoma from 2006 to 2021 were identified from an institutional registry. Patients undergoing primary surgical resection at our institution, ≥ 1 routine follow-up MRI, and pathologic diagnosis of ependymoma were included. Records were reviewed for demographic information, EOR, lesion characteristics, and pre-/post-operative neurologic symptoms. EOR was divided into 2 classifications: gross total resection (GTR) and subtotal resection (STR). Log-rank test was used to compare OS and PFS between patient groups.
RESULTS
Sixty-nine patients satisfied inclusion criteria, with 79.7% benefitting from GTR. The population was 56.2% male with average age of 45.7 years, and median follow-up duration of 58 months. Cox multivariate model demonstrated significant improvement in PFS when a GTR was attained (p <.001). Independently ambulatory patients prior to surgery had superior PFS (p <.001) and OS (p =.05). In univariate analyses, patients with a syrinx had improved PFS (p =.03) and were more likely to benefit from GTR (p =.01). Alternatively, OS was not affected by EOR (p =.78).
CONCLUSIONS
In this large, contemporary series of adult spinal ependymoma patients, we demonstrated improvements in PFS when GTR was achieved.
Topics: Humans; Male; Ependymoma; Female; Middle Aged; Adult; Spinal Cord Neoplasms; Neurosurgical Procedures; Progression-Free Survival; Follow-Up Studies; Retrospective Studies; Survival Rate; Young Adult; Aged; Prognosis; Adolescent
PubMed: 38438766
DOI: 10.1007/s11060-024-04623-4 -
World Neurosurgery Feb 2024Malignant soft tissue spinal canal tumors compromise 20% of all spinal neoplasms. They may be primary or metastatic lesions, originating from a diverse range of tissues... (Review)
Review
BACKGROUND
Malignant soft tissue spinal canal tumors compromise 20% of all spinal neoplasms. They may be primary or metastatic lesions, originating from a diverse range of tissues within and surrounding the spinal canal. These masses can present as diverse emergencies such as secondary cauda equina syndrome, vascular compromise, or syringomyelia. Interpretation of malignant soft tissue spinal canal tumors imaging is an essential for non-radiologists in the setting of emergencies. This task is intricate due to a great radiologic pattern overlap among entities.
METHODS
We present a step-by-step strategy that can guide nonradiologists identify a likely malignant soft tissue lesion in the spinal canal based on imaging features, as well as a review of the radiologic features of malignant soft tissue spinal canal tumors.
RESULTS
Diagnosis of soft tissue spinal canal malignancies starts with the identification of the lesion's spinal level and its relationship to the dura and medulla. The second step consists of characterizing it as likely-malignant based on radiological signs like a larger size, ill-defined margins, central necrosis, and/or increased vascularity. The third step is to identify additional imaging features such as intratumoral hemorrhage or cyst formation that can suggest specific malignancies. The physician can then formulate a differential diagnosis. The most encountered malignant soft tissue tumors of the spinal canal are anaplastic ependymomas, anaplastic astrocytomas, metastatic tumors, lymphoma, peripheral nerve sheath tumors, and central nervous system melanomas. A review of the imaging features of every type/subtype of lesion is presented in this work. Although magnetic resonance imaging remains the modality of choice for spinal tumor assessment, other techniques such as dynamic contrast agent-enhanced perfusion magnetic resonance imaging or diffusion-weighted imaging could guide diagnosis in specific situations.
CONCLUSIONS
In this review, diagnostic strategies for several spinal cord tumors were presented, including anaplastic ependymoma, metastatic spinal cord tumors, anaplastic and malignant astrocytoma, lymphoma, malignant peripheral nerve sheath tumors , and primary central nervous system melanoma. Although the characterization of spinal cord tumors can be challenging, comprehensive knowledge of imaging features can help overcome these challenges and ensure optimal management of spinal canal lesions.
PubMed: 38428809
DOI: 10.1016/j.wneu.2024.02.125