scholarly journals MRI-based diagnosis and treatment of pediatric brain tumors: is tissue sample always needed?

2021 ◽  
Author(s):  
Jehuda Soleman ◽  
Rina Dvir ◽  
Liat Ben-Sira ◽  
Michal Yalon ◽  
Frederick Boop ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Tissue Sample ◽  
Tumor Resection ◽  
Pediatric Brain Tumors ◽  
Mri Findings ◽  
Surgical Biopsy ◽  
Treatment Protocols ◽  
Treatment Regimens ◽  
Mri Features ◽  
Pediatric Brain

AbstractTraditional management of newly diagnosed pediatric brain tumors (PBTs) consists of cranial imaging, typically magnetic resonance imaging (MRI), and is frequently followed by tissue diagnosis, through either surgical biopsy or tumor resection. Therapy regimes are typically dependent on histological diagnosis. To date, many treatment regimens are based on molecular biology. The scope of this article is to discuss the role of diagnosis and further treatment of PBTs based solely on MRI features, in light of the latest treatment protocols. Typical MRI findings and indications for surgical biopsy of these lesions are described.

scholarly journals RONC-19. TWO CASES OF RE-IRRADIATION FOR LATE RECURRENT OR RADIATION-INDUCED TUMOR AFTER RADIATION THERAPY FOR PEDIATRIC BRAIN TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii459-iii459
Author(s):  
Takashi Mori ◽  
Shigeru Yamaguchi ◽  
Rikiya Onimaru ◽  
Takayuki Hashimoto ◽  
Hidefumi Aoyama
Keyword(s):  
Radiation Therapy ◽  
Brain Tumors ◽  
Tumor Resection ◽  
Intensity Modulated Radiation Therapy ◽  
Late Recurrence ◽  
Pediatric Brain Tumors ◽  
Suprasellar Region ◽  
Radiation Induced ◽  
Pediatric Brain

Abstract BACKGROUND As the outcome of pediatric brain tumors improves, late recurrence and radiation-induced tumor cases are more likely to occur, and the number of cases requiring re-irradiation is expected to increase. Here we report two cases performed intracranial re-irradiation after radiotherapy for pediatric brain tumors. CASE 1: 21-year-old male. He was diagnosed with craniopharyngioma at eight years old and underwent a tumor resection. At 10 years old, the local recurrence of suprasellar region was treated with 50.4 Gy/28 fr of stereotactic radiotherapy (SRT). After that, other recurrent lesions appeared in the left cerebellopontine angle, and he received surgery three times. The tumor was gross totally resected and re-irradiation with 40 Gy/20 fr of SRT was performed. We have found no recurrence or late effects during the one year follow-up. CASE 2: 15-year-old female. At three years old, she received 18 Gy/10 fr of craniospinal irradiation and 36 Gy/20 fr of boost to the posterior fossa as postoperative irradiation for anaplastic ependymoma and cured. However, a anaplastic meningioma appeared on the left side of the skull base at the age of 15, and 50 Gy/25 fr of postoperative intensity-modulated radiation therapy was performed. Two years later, another meningioma developed in the right cerebellar tent, and 54 Gy/27 fr of SRT was performed. Thirty-three months after re-irradiation, MRI showed a slight increase of the lesion, but no late toxicities are observed. CONCLUSION The follow-up periods are short, however intracranial re-irradiation after radiotherapy for pediatric brain tumors were feasible and effective.

scholarly journals SURG-24. NOVEL MALLEABLE FORCIPES FOR ENDOSCOPIC ASSISTED TECHNIQUE IN PEDIATRIC BRAIN TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii464-iii464
Author(s):  
Yukiko Nakahara ◽  
Hiroshi Ito ◽  
Fumitaka Yoshioka ◽  
Kohei Inoue ◽  
Atsushi Ogata ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Tumor Resection ◽  
Pediatric Brain Tumor ◽  
Pediatric Brain Tumors ◽  
Optical Devices ◽  
Surgical Instruments ◽  
Vascular Structure ◽  
Maximum Angle ◽  
Surgical Field ◽  
Pediatric Brain

Abstract Recent advances in optical devices and surgical instruments have been applied to neurosurgery. Even with modifications, one of the most serious risks is injury of neuronal and vascular structure caused by operation of surgical instruments in a narrow surgical field. Fixed instruments are not practical for pediatric brain tumor surgeries because the length of the curved or angled tip portion is limited because of the narrow entrance. We developed a novel malleable forceps to resolve the difficulties related to microsurgical procedures. The malleable forceps has two shafts with a sharp cup at the tip. The entire forceps was made of stainless steel, with a silver and nickel alloy inserted between 10 and 40 mm from the tip. In the alloy part, the surgeon can flex the forceps freely using a special cylinder. The special cylinder is useful to prevent from slipping of the cups of tip. The maximum angle that can be bent is 70 degrees vertically. We also developed a monoshaft malleable forceps. We used these flexible forcipes in the case of various pediatric brain tumors including craniopharyngioma. We performed tumor resection by anterior interhemispheric trans-lamina terminalis approach. After procedure of tumor resection using microscope, endoscope inserted around the pituitary stalk. The piece of calcified tumor could be easily removed without any complications. These forcipes can be deformed to an appropriate angle and can be applied to various cases, especially pediatric brain tumors.

scholarly journals Pediatric Brain Tumors: Innovative Genomic Information Is Transforming the Diagnostic and Clinical Landscape

2015 ◽  
Vol 33 (27) ◽  
pp. 2986-2998 ◽  
Author(s):  
Amar Gajjar ◽  
Daniel C. Bowers ◽  
Matthias A. Karajannis ◽  
Sarah Leary ◽  
Hendrik Witt ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Treatment Strategies ◽  
Genomic Analysis ◽  
Pediatric Brain Tumors ◽  
Diffuse Intrinsic Pontine Glioma ◽  
Collaborative Group ◽  
Treatment Protocols ◽  
New Information ◽  
Atypical Teratoid ◽  
Pediatric Brain

Pediatric neuro-oncology has undergone an exciting and dramatic transformation during the past 5 years. This article summarizes data from collaborative group and institutional trials that have advanced the science of pediatric brain tumors and survival of patients with these tumors. Advanced genomic analysis of the entire spectrum of pediatric brain tumors has heralded an era in which stakeholders in the pediatric neuro-oncology community are being challenged to reconsider their current research and diagnostic and treatment strategies. The incorporation of this new information into the next-generation treatment protocols will unleash new challenges. This review succinctly summarizes the key advances in our understanding of the common pediatric brain tumors (ie, medulloblastoma, low- and high-grade gliomas, diffuse intrinsic pontine glioma, and ependymoma) and some selected rare tumors (ie, atypical teratoid/rhabdoid tumor and CNS primitive neuroectodermal tumor). The potential impact of this new information on future clinical protocols also is discussed. Cutting-edge genomics technologies and the information gained from such studies are facilitating the identification of molecularly defined subgroups within patients with particular pediatric brain tumors. The number of evaluable patients in each subgroup is small, particularly in the subgroups of rare diseases. Therefore, international collaboration will be crucial to draw meaningful conclusions about novel approaches to treating pediatric brain tumors.

scholarly journals SURG-10. SPECTROSCOPIC MEASUREMENT OF 5-ALA-INDUCED INTRACELLULAR PROTOPORPHYRIN IX IN PEDIATRIC BRAIN TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii462-iii463
Author(s):  
Michael Schwake ◽  
Sadahiro Kaneko ◽  
Eric Suero Morina ◽  
Walter Stummer
Keyword(s):  
Brain Tumors ◽  
Fluorescence Intensity ◽  
Laboratory Data ◽  
Tumor Resection ◽  
Pediatric Brain Tumors ◽  
Fluorescence Signal ◽  
Normal Brain ◽  
Diffuse Astrocytoma ◽  
Intracellular Fluorescence ◽  
Pediatric Brain

Abstract OBJECTIVE 5-ALA guided resection of glioma in adults enables better delineation between tumor and normal brain, allowing improved resection and improved patients’ outcome. Recently, several reports were published regarding 5-ALA for resection of pediatric brain tumors. The aim of the study was to determine the intracellular fluorescence of PPIX in pediatric brain tumors by hyperspectral imaging and to compare it with visually observed intraoperative fluorescence. METHODS 5-ALA was administered orally four hours prior to surgery. During tumor resection the surgeon assessed the fluorescence signal to be strong, weak or absent. Subsequently, fluorescence intensity of samples was measured via spectroscopy. In addition, clinical data, imaging and laboratory data were analyzed. RESULTS Eleven children (1–16 years) were operated. Tumor entities included: three medulloblastomas, two pilocytic astrocytomas (PA), two anaplastic ependymomas and one diffuse astrocytoma, anaplastic astrocytoma, pilomyxoid astrocytoma and anaplastic pleomorphic xanthoastrocytoma. Strong fluorescence was visible in all anaplastic tumors and one PA; one PA demonstrated weak fluorescence. Visible fluorescence was strongly associated with intracellular fluorescence intensity and PPIX concentration (P<0.05). Within all tumors with visible fluorescence the intracellular PPIX concentration was greater than 4 µg/ml. Except for moderate and transient elevation of liver enzymes, no 5-ALA related adverse events were reported. CONCLUSION We demonstrate a strong association between intraoperative observations and spectrometric measurements of PPIX fluorescence in tumor tissue. As in former studies, fluorescence signal was more commonly observed in malignant glial tumors. Further prospective controlled trials should be conducted to investigate the feasibility of 5-ALA guided resection of pediatric brain tumors.

scholarly journals IMG-13. MRI-BASED RADIOMICS PROGNOSTIC MARKERS OF POSTERIOR FOSSA EPENDYMOMA

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii357-iii357
Author(s):  
Lydia Tam ◽  
Derek Yecies ◽  
Michelle Han ◽  
Sebastien Toescu ◽  
Jason Wright ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Posterior Fossa ◽  
Clinical Features ◽  
Cox Regression ◽  
Pediatric Brain Tumors ◽  
Clinical Variable ◽  
Post Contrast ◽  
First Order ◽  
Mri Features ◽  
Pediatric Brain

Abstract PURPOSE Posterior fossa ependymomas (PFE) are common pediatric brain tumors often assessed with MRI before surgery. Advanced radiomic analysis show promise in stratifying risk and outcome in other pediatric brain tumors. Here, we extracted high-dimensional MRI features to identify prognostic, image-based, radiomics markers of PFE and compared its performance to clinical variables. METHODS 93 children from five centers (median age=3.3yrs; 59 males; mean PFS=50mos) were included. Tumor volumes were manually contoured on T1-post contrast and T2-weighted MRI for PyRadiomics feature extraction. Features include first-order statistics, size, shape, and texture metrics calculated on the original, log-sigma, and wavelet transformed images. Progression free survival (PFS) served as outcome. 10-fold cross-validation of a LASSO Cox regression was used to predict PFS. Model performance was analyzed and concordance metric (C) was determined using clinical variable (age at diagnosis and sex) only, radiomics only, and radiomics plus clinical variable. RESULTS Six radiomic features were selected (all T1): 1 first-order kurtosis (log-sigma) and 5 texture features (3 wavelet, 2 original). This model demonstrated significantly higher performance than a clinical model alone (C: 0.69 vs 0.58, p<0.001). Adding clinical features to the radiomic features didn’t improve prediction (p=0.67). For patients with molecular subtyping (n=48), adding this feature to the clinical plus radiomics models significantly improved performance over clinical features alone (C = 0.79 vs. 0.66, p=0.02). Further validation and model refinement with additional datasets are ongoing. CONCLUSION Our pilot study shows potential role for MRI-based radiomics and machine learning for PFE risk stratification and as radiographic biomarkers.

scholarly journals Typical Pediatric Brain Tumors Occurring in Adults—Differences in Management and Outcome

Biomedicines ◽  
2021 ◽  
Vol 9 (4) ◽  
pp. 356
Author(s):  
Ladina Greuter ◽  
Raphael Guzman ◽  
Jehuda Soleman
Keyword(s):  
Overall Survival ◽  
Brain Tumors ◽  
Pilocytic Astrocytoma ◽  
Pediatric Brain Tumors ◽  
Adult Brain ◽  
Treatment Regimens ◽  
Specific Distribution ◽  
Specific Outcome ◽  
Adults And Children ◽  
Pediatric Brain

Adult brain tumors mostly distinguish themselves from their pediatric counterparts. However, some typical pediatric brain tumors also occur in adults. The aim of this review is to describe the differences between classification, treatment, and outcome of medulloblastoma, pilocytic astrocytoma, and craniopharyngioma in adults and children. Medulloblastoma is a WHO IV posterior fossa tumor, divided into four different molecular subgroups, namely sonic hedgehog (SHH), wingless (WNT), Group 3, and Group 4. They show a different age-specific distribution, creating specific outcome patterns, with a 5-year overall survival of 25–83% in adults and 50–90% in children. Pilocytic astrocytoma, a WHO I tumor, mostly found in the supratentorial brain in adults, occurs in the cerebellum in children. Complete resection improves prognosis, and 5-year overall survival is around 85% in adults and >90% in children. Craniopharyngioma typically occurs in the sellar compartment leading to endocrine or visual field deficits by invasion of the surrounding structures. Treatment aims for a gross total resection in adults, while in children, preservation of the hypothalamus is of paramount importance to ensure endocrine development during puberty. Five-year overall survival is approximately 90%. Most treatment regimens for these tumors stem from pediatric trials and are translated to adults. Treatment is warranted in an interdisciplinary setting specialized in pediatric and adult brain tumors.

scholarly journals A pediatric brain tumor atlas of genes deregulated by somatic genomic rearrangement

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Yiqun Zhang ◽  
Fengju Chen ◽  
Lawrence A. Donehower ◽  
Michael E. Scheurer ◽  
Chad J. Creighton
Keyword(s):  
Brain Tumor ◽  
Brain Tumors ◽  
Tyrosine Kinases ◽  
Pediatric Brain Tumor ◽  
Pediatric Brain Tumors ◽  
Altered Expression ◽  
Critical Pathways ◽  
Cis Regulation ◽  
Or Gene ◽  
Pediatric Brain

AbstractThe global impact of somatic structural variants (SSVs) on gene expression in pediatric brain tumors has not been thoroughly characterised. Here, using whole-genome and RNA sequencing from 854 tumors of more than 30 different types from the Children’s Brain Tumor Tissue Consortium, we report the altered expression of hundreds of genes in association with the presence of nearby SSV breakpoints. SSV-mediated expression changes involve gene fusions, altered cis-regulation, or gene disruption. SSVs considerably extend the numbers of patients with tumors somatically altered for critical pathways, including receptor tyrosine kinases (KRAS, MET, EGFR, NF1), Rb pathway (CDK4), TERT, MYC family (MYC, MYCN, MYB), and HIPPO (NF2). Compared to initial tumors, progressive or recurrent tumors involve a distinct set of SSV-gene associations. High overall SSV burden associates with TP53 mutations, histone H3.3 gene H3F3C mutations, and the transcription of DNA damage response genes. Compared to adult cancers, pediatric brain tumors would involve a different set of genes with SSV-altered cis-regulation. Our comprehensive and pan-histology genomic analyses reveal SSVs to play a major role in shaping the transcriptome of pediatric brain tumors.

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