scholarly journals RARE-12. VASCULOPATHY IN PEDIATRIC CRANIOPHARYNGIOMA PATIENTS TREATED WITH SURGERY AND RADIOTHERAPY

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii444-iii444
Author(s):  
John Lucas ◽  
David LeVine ◽  
Yousef Ismael ◽  
Chi-Yang Hsu ◽  
Kaleb Darrow ◽  
...  
Keyword(s):  
Vascular Injury ◽  
Clinical Characteristics ◽  
Pediatric Brain Tumor ◽  
High Dose ◽  
Normal Brain ◽  
Radiotherapy Field ◽  
Clinically Significant ◽  
Radiotherapy Dose ◽  
The Relationship ◽  
Pediatric Brain

Abstract PURPOSE As much as 40% of pediatric brain tumor patients will experience varied levels of Vasculopathy (VS), however few predictive factors have been described. Here we describe the type and timing of VS and explore the relationship between treatment modality and the timing, location, and distribution of VS. METHODS 94 pediatric Craniopharyngioma patients underwent surgery and proton radiotherapy. Pre- and post-treatment imaging, cumulative physical and biological proton dose maps, clinical characteristics, and measures of dyslipidemia were evaluated. MR and MRAs were evaluated for pre- and post-radiotherapy VS (type, workup, location, and severity). VS events were segmented and described according to their normal brain region, and vascular territory. RESULTS 47 patients were found to have 154 confirmed VS of varying severity with a median time to event of 3.41 years 95% CI 3.08–3.88. 22% (N=21) of patients had ≥1 pre-existing instances of VS and 26.6% (N=25) had a dyslipidemia at diagnosis. Forty-six (48.9%) patients had evidence of VS post-RT with 9.5% (N=9) being clinically significant. Aspirin was recommended in 10.6% (N=10) patients. Only 4 (4.2%) patients required revascularization. Clinical characteristics were not predictive of VS. An increased frequency of VS were observed along the operative corridor and high-dose radiotherapy field. CONCLUSIONS VS often precedes radiotherapy necessitating appropriate baseline imaging. Surgery type and extent are interrelated to the risk for radiotherapy-induced VS. While the spatial radiotherapy dose distribution approximated most vascular injury events, it was not all-inclusive. Spatial modeling of biological and physical dose may offer insights into therapy related vascular injury.

scholarly journals Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature

2011 ◽  
Vol 30 (1) ◽  
pp. E2 ◽  
Author(s):  
Sunil Manjila ◽  
Abhishek Ray ◽  
Yin Hu ◽  
Dan X. Cai ◽  
Mark L. Cohen ◽  
...  
Keyword(s):  
Treatment Strategies ◽  
Pediatric Brain Tumor ◽  
High Dose ◽  
Poor Clinical Outcome ◽  
Embryonal Tumors ◽  
Embryonal Tumor ◽  
Hematopoietic Stem ◽  
Stem Cell Rescue ◽  
Neuroepithelial Cells ◽  
Pediatric Brain

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently identified variant of primitive neuroectodermal tumor, with fewer than 50 cases reported in the literature to date. Histologically, this tumor has features of ependymoblastoma and neuroblastoma, demonstrating areas of fine fibrillary neuropil intermingled with ependymoblastic rosettes and zones of undifferentiated neuroepithelial cells. However, ETANTR is distinguished pathologically from other embryonal tumors by the striking abundance of neuropil. Clinically, ETANTRs have shown high malignant potential and poor clinical outcome despite aggressive treatment. The authors describe 2 illustrative surgical cases of ETANTR, one involving the longest reported survival in the literature to date. The other had a poor outcome despite high-dose adjuvant chemotherapy with sequential autologous hematopoietic stem cell rescue. The authors review the natural history and treatment strategies available for this unusual malignant pediatric brain tumor.

scholarly journals OTME-3. Dissection of the role of stromal microenvironment and tumor-TME crosstalk in pediatric brain cancer

2021 ◽  
Vol 3 (Supplement_2) ◽  
pp. ii13-ii14
Author(s):  
Aniello Federico ◽  
Marcel Kool
Keyword(s):  
Cell Communication ◽  
Pediatric Brain Tumor ◽  
Therapeutic Strategies ◽  
Human Tumors ◽  
Normal Brain ◽  
Malignant Cells ◽  
Sequencing Data ◽  
Communication Analysis ◽  
Type Assignment ◽  
Pediatric Brain

Abstract Brain tumors are the deadliest malignancies that occur during childhood and strong efforts are required to develop innovative therapeutic strategies. The intrinsic capacity of malignant cells to organize, shape and exploit the surrounding environment where they develop (tumor microenvironment, TME), has not been fully elucidated for pediatric brain cancers yet. Here, we exploited a multi-omic approach to define the TME cell populations and their contributions in the most common pediatric brain tumor entities, such as medulloblastomas and ependymomas. Analysis of single-cell RNA sequencing data of human tumors resulted in the identification of heterogeneous populations of non-malignant cells present in the TME. In particular, re-clustering and marker-based cell type assignment strategies allowed to define a broad range of immune and stromal subclasses showing distinctive expression signatures reflecting variegated functional roles. By cross-matching the tumor data with normal brain expression atlases, we could further refine the annotation of the newly identified stromal functional subpopulations and define the “tumor-associated” marker signatures of genes exclusively enriched in stromal cells within the TME, linked to immune activation, cell adhesion and cytokine regulation pathways. Bulk transcriptomic data of human tumors and matching patient-derived xenografts (PDXs) showed that a group of secreted stromal factors acting as regulators of tumorigenic mechanisms, such as IGF2 and COL4A1, are lost after xenografting and replaced by the host murine microenvironment, suggesting that tumor cells are involved in paracrine and bivalent crosstalk with TME cells, impacting on tumor cell growth and progression. Finally, bulk deconvolution and cell-cell communication analysis were exploited to define, respectively, the stromal cell proportions and the key factors involved in the tumor-TME crosstalk; this latter can be considered as possible targets for tailored and more specific anti-tumor therapeutic strategies.

scholarly journals NCOG-20. GENETIC MARKERS AND CLINICAL CHARACTERISTICS RELATED TO NEUROCOGNITIVE OUTCOMES OF PEDIATRIC BRAIN TUMOR PATIENTS

2017 ◽  
Vol 19 (suppl_6) ◽  
pp. vi142-vi142
Author(s):  
Cassie Kline-Nunnally ◽  
Erin Felton ◽  
Caleb Edwards ◽  
Heather Fullerton ◽  
Joseph Torkildson ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Genetic Markers ◽  
Clinical Characteristics ◽  
Pediatric Brain Tumor ◽  
Tumor Patients ◽  
Neurocognitive Outcomes ◽  
Pediatric Brain

scholarly journals SURG-17. CLINICAL CHARACTERISTICS AND OUTCOMES OF EPILEPSY-RELATED BRAIN TUMOR IN CHILDREN

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii463-iii463
Author(s):  
Kenichi Usami ◽  
Keita Terashima ◽  
Yuichi Abe ◽  
Chikako Kiyotani ◽  
Hideki Ogiwara
Keyword(s):  
Brain Tumor ◽  
Clinical Characteristics ◽  
Tumor Resection ◽  
Intractable Epilepsy ◽  
Pediatric Brain Tumor ◽  
Pediatric Epilepsy ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Initial Surgery ◽  
Age At Surgery ◽  
Pediatric Brain

Abstract OBJECTIVE Epilepsy is one of the earliest symptoms in pediatric brain tumor. Gross total resection (GTR) of the tumor does not necessarily achieve seizure free, therefore it is controversial whether surrounding epileptic foci should be resected at the initial surgery. The aims of this study are to report clinical characteristics and outcome of pediatric epilepsy-related brain tumor (ERBT) and to discuss treatment strategy. METHODS Subjects were children less than 18 years old who underwent surgery for ERBT. Patients in whom epilepsy had been controlled before surgery were excluded. Data were collected from medical record and retrospectively reviewed. RESULTS Twenty-one children (8 boys and 13 girls) were analyzed in this study. The mean age at surgery was 6.8 years. Tumor was astrocytic tumor in 10, gangliogioma in 4 and dysembryoplastic neuroepithelial tumor in 3. Intracranial subdural electrodes were placed prior to tumor resection in 5 cases. GTR was achieved in 14 (67%). Seizure free was achieved in 15 (71.4%). GTR was significantly associated with seizure free (p=0.002). CONCLUSION In most of ERBT, seizure free can be achieved by lesionectomy alone. However, the resection of surrounding epileptic foci is required in some cases. Detailed examinations to detect the epileptic foci should be performed in ERBT, particularly in case of drug-resistant intractable epilepsy.

Time to diagnosis and clinical characteristics in pediatric brain tumor patients

2020 ◽  
Vol 36 (9) ◽  
pp. 2047-2054 ◽  
Author(s):  
Koji Hirata ◽  
Ai Muroi ◽  
Takao Tsurubuchi ◽  
Hiroko Fukushima ◽  
Ryoko Suzuki ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Clinical Characteristics ◽  
Pediatric Brain Tumor ◽  
Tumor Patients ◽  
Time To Diagnosis ◽  
Pediatric Brain

scholarly journals Targeting MYCN in Molecularly Defined Malignant Brain Tumors

2021 ◽  
Vol 10 ◽  
Author(s):  
Anna Borgenvik ◽  
Matko Čančer ◽  
Sonja Hutter ◽  
Fredrik J. Swartling
Keyword(s):  
Brain Tumor ◽  
Brain Tumors ◽  
Pediatric Brain Tumor ◽  
Protein Stabilization ◽  
Adult Brain ◽  
Normal Brain ◽  
Malignant Brain Tumors ◽  
Indirect Measures ◽  
Tumor Types ◽  
Pediatric Brain

Misregulation of MYC genes, causing MYC overexpression or protein stabilization, is frequently found in malignant brain tumors highlighting their important roles as oncogenes. Brain tumors in children are the most lethal of all pediatric malignancies and the most common malignant primary adult brain tumor, glioblastoma, is still practically incurable. MYCN is one of three MYC family members and is crucial for normal brain development. It is associated with poor prognosis in many malignant pediatric brain tumor types and is focally amplified in specific adult brain tumors. Targeting MYCN has proved to be challenging due to its undruggable nature as a transcription factor and for its importance in regulating developmental programs also in healthy cells. In this review, we will discuss efforts made to circumvent the difficulty of targeting MYCN specifically by using direct or indirect measures to treat MYCN-driven brain tumors. We will further consider the mechanism of action of these measures and suggest which molecularly defined brain tumor patients that might benefit from MYCN-directed precision therapies.

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