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Child's Nervous System : ChNS :... Feb 2023The occipital interhemispheric transtentorial (OITT) approach is frequently used for accessing the pineal region. There are scarce reports of using the OITT to access...
BACKGROUND
The occipital interhemispheric transtentorial (OITT) approach is frequently used for accessing the pineal region. There are scarce reports of using the OITT to access superior cerebellar lesions. This approach affords the patient several advantages over traditional posterior fossa approaches.
PURPOSE
This study is to describe and evaluate clinical outcomes in a single surgeon case series of the OITT approach for pediatric patients with lesions of the superior cerebellum.
METHODS
All pediatric patients who underwent an OITT craniotomy for a superior cerebellar lesion by a single surgeon over a 5-year period were included in this retrospective analysis. Patient demographics and clinical data were collected.
RESULTS
Thirteen pediatric patients were identified. Cases included twelve tumors and one arteriovenous malformation. Gross total resection was achieved in 92% of cases. No patients developed posterior fossa syndrome. Two patients had transient homonymous hemianopsia that resolved by 1 month post-operatively. There were no permanent neurological deficits.
CONCLUSION
For superomedial cerebellar lesions presenting to the tentorial surface of the superior cerebellum in patients with normal to steep tentorial angles, the OITT approach is effective and safe. This approach has a low risk of posterior fossa syndrome and permanent visual deficits when applied appropriately. Patient selection is critical for maximizing the advantages of the OITT for superior cerebellar lesions.
Topics: Humans; Child; Retrospective Studies; Cerebellum; Craniotomy; Dura Mater; Pineal Gland; Neurosurgical Procedures
PubMed: 36380052
DOI: 10.1007/s00381-022-05761-8 -
La Clinica Terapeutica 2022Histograms can be determined throughout tumors, relying partly on existing tumor microstructure knowledge and the sampling effect from area of interest analyses. We...
PURPOSE
Histograms can be determined throughout tumors, relying partly on existing tumor microstructure knowledge and the sampling effect from area of interest analyses. We aimed to investigate the impact of ADC histogram parameters in discriminating medulloblastoma, ependymoma, and pilocytic astrocytoma.
METHODS
This study received approval from the Institutional Ethics Review Committee of Children's Hospital 02. Processes were conducted according to relevant laws and regulations, and requirements for written informed consent were fulfilled. The study involved 24 patients at Children's Hospital 02 from February-December 2019. Group 1 included 12 children with medulloblastoma, group 2 included 5 with ependymoma, and group 3 included 7 with pilocytic astrocytoma. All patients underwent MRI followed by surgery or biopsy to obtain histopathological confirmations.
RESULTS
Our analysis indicated that AUC, sensitivity, and specificity were 96.7%, 91.7%, and 100%, respectively when ADCkurtosis (cut-off point = 2.34) was taken to differentiate between medulloblasto-mas and ependymomas. To distinguish between medulloblastomas and pilocytic astrocytomas, the cut-off points of ADCmean, ADCmedian, ADCmax, ADCmin, rADCmean, rADCmax, and rADCmin of 0.985, 0.910, 1.305, 0.710, 1.349, 1.738, and 1.251, were taken respectively with AUC, sensitivity, and specificity elicited at 100%. To discriminate between ependymomas and pilocytic astrocytomas, the cut-off points of ADCmean, ADCmedian, ADCmax, ADCmin, rADCmean, rADC-median, rADCmax and rADCmin were 1.010, 0.930, 1.270, 0.735, 1.346, 1.324, 1.676, and 1.273, respectively, with AUC, sensitivity, and specificity at 100%.
CONCLUSION
ADC histograms can facilitate differentiation among juvenile medulloblastoma, ependymoma, and pilocytic astrocytoma, providing reliable, objective evidence of tumor differentiation.
Topics: Astrocytoma; Cerebellar Neoplasms; Child; Diffusion Magnetic Resonance Imaging; Ependymoma; Humans; Medulloblastoma; Retrospective Studies
PubMed: 35857056
DOI: 10.7417/CT.2022.2448 -
Neuro-oncology Advances 2022Pediatric gliomas comprise a diverse set of brain tumor entities that have substantial long-term ramifications for patient survival and quality of life. However, the...
BACKGROUND
Pediatric gliomas comprise a diverse set of brain tumor entities that have substantial long-term ramifications for patient survival and quality of life. However, the study of these tumors is currently limited due to a lack of authentic models. Additionally, many aspects of pediatric brain tumor biology, such as tumor cell invasiveness, have been difficult to study with currently available tools. To address these issues, we developed a synthetic extracellular matrix (sECM)-based culture system to grow and study primary pediatric brain tumor cells.
METHODS
We developed a brain-like sECM material as a supportive scaffold for the culture of primary, patient-derived pediatric glioma cells and established patient-derived cell lines. Primary juvenile brainstem-derived murine astrocytes were used as a feeder layer to support the growth of primary human tumor cells.
RESULTS
We found that our culture system facilitated the proliferation of various primary pediatric brain tumors, including low-grade gliomas, and enabled ex vivo testing of investigational therapeutics. Additionally, we found that tuning this sECM material allowed us to assess high-grade pediatric glioma cell invasion and evaluate therapeutic interventions targeting invasive behavior.
CONCLUSION
Our sECM culture platform provides a multipurpose tool for pediatric brain tumor researchers that enables both a wide breadth of biological assays and the cultivation of diverse tumor types.
PubMed: 35669012
DOI: 10.1093/noajnl/vdac049 -
Diagnostics (Basel, Switzerland) Apr 2022Primary brain tumors are the most common solid neoplasms in children and a leading cause of mortality in this population. MRI plays a central role in the diagnosis,... (Review)
Review
Primary brain tumors are the most common solid neoplasms in children and a leading cause of mortality in this population. MRI plays a central role in the diagnosis, characterization, treatment planning, and disease surveillance of intracranial tumors. The purpose of this review is to provide an overview of imaging methodology, including conventional and advanced MRI techniques, and illustrate the MRI appearances of common pediatric brain tumors.
PubMed: 35454009
DOI: 10.3390/diagnostics12040961 -
Translational Oncology Jun 2022Brain tumors are the leading cause of cancer-related deaths in children. Tailored therapies need preclinical brain tumor models representing a wide range of molecular...
Brain tumors are the leading cause of cancer-related deaths in children. Tailored therapies need preclinical brain tumor models representing a wide range of molecular subtypes. Here, we adapted a previously established brain tissue-model to fresh patient tumor cells with the goal of establishing3D in vitro culture conditions for each tumor type.Wereported our findings from 11 pediatric tumor cases, consisting of three medulloblastoma (MB) patients, three ependymoma (EPN) patients, one glioblastoma (GBM) patient, and four juvenile pilocytic astrocytoma (Ast) patients. Chemically defined media consisting of a mixture of pro-neural and pro-endothelial cell culture medium was found to support better growth than serum-containing medium for all the tumor cases we tested. 3D scaffold alone was found to support cell heterogeneity and tumor type-dependent spheroid-forming ability; both properties were lost in 2D or gel-only control cultures. Limited in vitro models showed that the number of differentially expressed genes between in vitro vs. primary tissues, are 104 (0.6%) of medulloblastoma, 3,392 (20.2%) of ependymoma, and 576 (3.4%) of astrocytoma, out of total 16,795 protein-coding genes and lincRNAs. Two models derived from a same medulloblastoma patient clustered together with the patient-matched primary tumor tissue; both models were 3D scaffold-only in Neurobasal and EGM 1:1 (v/v) mixture and differed by a 1-mo gap in culture (i.e., 6wk versus 10wk). The genes underlying the in vitrovs. in vivo tissue differences may provide mechanistic insights into the tumor microenvironment. This study is the first step towards establishing a pipeline from patient cells to models to personalized drug testing for brain cancer.
PubMed: 35381525
DOI: 10.1016/j.tranon.2022.101407 -
Surgical Neurology International 2021Many patients with spinal juvenile pilocytic astrocytoma can experience prolonged remission after resection. However, some reports suggest that pregnancy may be...
BACKGROUND
Many patients with spinal juvenile pilocytic astrocytoma can experience prolonged remission after resection. However, some reports suggest that pregnancy may be associated with progression.
CASE DESCRIPTION
The authors provide an image report highlighting a case of rapid and aggressive transformation of an intramedullary astrocytoma of the cervical spine in a pregnant patient. Over the course of 1 year, the lesion progressed from a juvenile pilocytic astrocytoma to an anaplastic astrocytoma. Genetic testing revealed mutations associated with aggressive behavior.
CONCLUSION
The case and associated imaging demonstrate the importance of close neurologic monitoring and counseling regarding risk of progression in pregnant patients with spinal gliomas.
PubMed: 34621581
DOI: 10.25259/SNI_759_2021 -
Pediatrics in Review Sep 2021Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but... (Review)
Review
Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but so too with their adult equivalents. Some of the most common diseases are autoimmune (ie, multiple sclerosis, acute disseminated encephalomyelitis, and acute transverse myelitis), congenital (ie, dysraphism with spina bifida, split cord malformation, and tethered cord syndrome), tumor (ie, juvenile pilocytic astrocytoma, ependymoma, and hem-angioblastoma), and vascular (ie, cavernous malformations, arteriovenous malformations, and dural arteriovenous fistulas) in nature. These each require their own niche treatment paradigm and prognosis. Furthermore, presentation of different spinal cord diseases in children can be difficult to discern without epidemiologic and imaging data. Interpretation of these data is crucial to facilitating a timely and accurate diagnosis. Correspondingly, the aim of this review was to highlight the most pertinent features of the most common spinal cord diseases in the pediatric population.
Topics: Adult; Child; Encephalomyelitis, Acute Disseminated; Humans; Magnetic Resonance Imaging; Myelitis, Transverse; Neural Tube Defects; Spinal Cord; Spinal Cord Diseases; Spinal Dysraphism
PubMed: 34470868
DOI: 10.1542/pir.2020-000661 -
Journal of Neurosurgical Sciences Jun 2023Juvenile pilocytic astrocytoma (JPA) typically follows an indolent clinical course. The first-line treatment for most JPAs is surgical resection. However, a gross total...
BACKGROUND
Juvenile pilocytic astrocytoma (JPA) typically follows an indolent clinical course. The first-line treatment for most JPAs is surgical resection. However, a gross total resection may not be feasible for deep-seated lesions and/or infiltrative tumors, leading to multimodal treatment approaches that may be complicated by patient age and tumor location. Despite the prevalence of pediatric JPAs, there is no single approach to treating progressive disease.
METHODS
We investigated the multifaceted management of progressive JPAs through a retrospective analysis of JPAs treated at a single center over an 18-year period (1998-2016). All cases were categorized according to location, whether supratentorial or infratentorial, and for each case we calculated the number of interventions and the time between interventions.
RESULTS
We identified a total of 40 JPAs, (11 supratentorial, 29 infratentorial). Total number of interventions among all supratentorial JPA patients was 21 (average 2 interventions/patient). The total number of interventions among infratentorial JPAs was 40 (average 1.4 interventions/patient).
CONCLUSIONS
Treatment of progressive JPA is variable and may require numerous surgeries and adjuvant therapies.
Topics: Humans; Child; Brain Neoplasms; Retrospective Studies; Astrocytoma
PubMed: 33870664
DOI: 10.23736/S0390-5616.21.05169-9 -
Surgical Neurology International 2021Low-grade gliomas (LGG) are described by the World Health Organization as Grades I and II. Among LGGs, the most common primary brain tumor is pilocytic astrocytoma (PA)...
BACKGROUND
Low-grade gliomas (LGG) are described by the World Health Organization as Grades I and II. Among LGGs, the most common primary brain tumor is pilocytic astrocytoma (PA) and carries an excellent prognosis when treated with complete surgical resection. Cases, in which this is not possible, are associated with less favorable outcomes and worse progression-free survival.
CASE DESCRIPTION
This report describes a case of a 22-year-old male, who presented with progression of a primary brainstem tumor previously treated with stereotactic radiosurgery and chemotherapy. Patient underwent surgical exploration and was diagnosed with juvenile PA, but debulking was limited by the very dense and fibrous tumor. Complete surgical resection was not possible at this time. Despite efforts to treat with chemotherapy, the patient presented a year later with clinical deterioration and severe neurologic deficits, prompting surgical re-exploration. During the second operation, the tumor was found to have undergone very significant softening in consistency, allowing for gross total resection (GTR).
CONCLUSION
Aggressive treatment of brainstem LGG should be pursued whenever possible, given its generally favorable prognosis. Repeat microsurgical resection, even with a different approach, might be reasonable and safe. Finally, chemotherapy may be associated with changes in the tumor consistency that can render previously unresectable lesions amenable to successful aggressive resection.
PubMed: 33500827
DOI: 10.25259/SNI_594_2020 -
Endocrine Practice : Official Journal... Oct 2020To explore incidental findings on brain magnetic resonance imaging (MRI) studies of pediatric patients referred due to endocrine disorders. (Observational Study)
Observational Study
OBJECTIVE
To explore incidental findings on brain magnetic resonance imaging (MRI) studies of pediatric patients referred due to endocrine disorders.
METHODS
A retrospective, observational study conducted in a tertiary referral center. The neuroimaging database of 17,445 brain MRI studies of 11,011 pediatric patients were searched for cases with endocrine referrals and without medical history of malignancy, genetic syndromes, and/or neurologic comorbidities. This database was linked to the pediatric neurosurgical database. Clinical data were retrieved from medical files.
RESULTS
In total, 524 patients (50.2% males, mean age 8.5±3.5 years) were referred to brain MRI due to growth disturbances (n = 313), pubertal disorders (n = 183), prolactin hypersecretion (n = 18), central diabetes insipidus (n = 8), and obesity (n = 1). Incidental findings were found in 128 (24.4%) cases. Chiari type 1 malformation was more prevalent in patients with growth disturbances (P<.001). Small pituitary cysts were observed in 20 (3.8%) patients, and pineal cysts in 25 (4.8%) patients, mostly girls (68%, P<.001). White matter lesions were diagnosed in 30 (5.7%) patients, none with clinical evidence of neurologic disease. Brain asymmetry without clinical significance and developmental venous anomalies were observed in 14 (2.7%) and 8 (1.5%) patients, respectively. Twelve patients were diagnosed with intracranial tumors, and 5 required surgical intervention for a histopathologic diagnosis of juvenile pilocytic astrocytoma (n = 3), choroid plexus papilloma (n = 1), or inconclusive (n = 1). The rest were managed conservatively.
CONCLUSION
Incidental findings on brain MRIs of pediatric patients referred by endocrinologists are common and raise dilemmas. The spectrum ranges from structural disruptions to tumors. Decision-making is individualized and patient-centered.
Topics: Brain; Child; Child, Preschool; Female; Humans; Incidental Findings; Magnetic Resonance Imaging; Male; Neuroimaging; Retrospective Studies
PubMed: 33471712
DOI: 10.4158/EP-2020-0208