Medulloblastoma

Millard, Nathan E.; Braganca, Kevin C. De

doi:10.1177/0883073815600866

Cited by 210 publications

(255 citation statements)

References 114 publications

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“…MB is the most common malignant brain tumor in children [9] with a culminating incidence before the age of five [10]. They are treated using combined therapeutic approaches, which include surgical resection as well as radio-and chemotherapy [11]. Although these treatments strongly improve overall survival rates of MB patients, they are extremely aggressive and devastating for these young patients.…”

Section: Introductionmentioning

confidence: 99%

Splicing Dysregulation as Oncogenic Driver and Passenger Factor in Brain Tumors

Bielli

Pagliarini

Pieraccioli

et al. 2019

Cells

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Brain tumors are a heterogeneous group of neoplasms ranging from almost benign to highly aggressive phenotypes. The malignancy of these tumors mostly relies on gene expression reprogramming, which is frequently accompanied by the aberrant regulation of RNA processing mechanisms. In brain tumors, defects in alternative splicing result either from the dysregulation of expression and activity of splicing factors, or from mutations in the genes encoding splicing machinery components. Aberrant splicing regulation can generate dysfunctional proteins that lead to modification of fundamental physiological cellular processes, thus contributing to the development or progression of brain tumors. Herein, we summarize the current knowledge on splicing abnormalities in brain tumors and how these alterations contribute to the disease by sustaining proliferative signaling, escaping growth suppressors, or establishing a tumor microenvironment that fosters angiogenesis and intercellular communications. Lastly, we review recent efforts aimed at developing novel splicing-targeted cancer therapies, which employ oligonucleotide-based approaches or chemical modulators of alternative splicing that elicit an impact on brain tumor biology.

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Section: Introductionmentioning

confidence: 99%

Splicing Dysregulation as Oncogenic Driver and Passenger Factor in Brain Tumors

Bielli

Pagliarini

Pieraccioli

et al. 2019

Cells

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“…MB is frequently located in the posterior fossa. Radiotherapy of the craniospinal irradiation (CSI) techniques and chemotherapy are the standard treatments after operation of medulloblastoma [1].…”

Section: Introductionmentioning

confidence: 99%

Recurrence pattern and Survival analysis in patients undergoing radiotherapy IMRT technique for medulloblastoma

Chang

Wang

et al. 2020

Preprint

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Objective The technique of radiotherapy is associated with the recurrence pattern and prognosis of postoperative treatment for medulloblastoma. Our aim was to assess recurrence patterns and survival in patients with medulloblastoma between three-dimensional conformation (3D-CRT) and intensity modulated radiotherapy (IMRT). To analyze the factors affecting the prognosis, such as the use of 3D-CRT or IMRT, the time interval of surgical and radiotherapy, whether there is chemotherapy and chemotherapy regimen.Methods In this study, 36 patients with medulloblastoma were conducted investigating and data were analyzed from May 2009 to September 2017. The median survival of each group was calculated for different clinical stages,pathological types and treatment methods. The PFS and OS for patients curves were derived by Kaplan-Meier estimation, and which were calculated using the Cox proportional hazards model between the IMRT and 3D-CRT treatment groups.Results Up to the follow-up date, 7 of the 9 patients in the 3D-CRT group had recurrence and 9 of the 27 patients in the IMRT group had recurrence. The 2-year and 5-year PFS of the IMRT group were 74.1% and 65.8%, and which of the 3D-CRT group were 55.6% and 22.2%, respectively. The PFS was significantly higher for patients treated with IMRT compared with 3D-CRT (P<0.05). The 2-year overall survival rates of the IMRT group compared with the 3D-CRT group were the same, the 5-year overall survival rate of the IMRT group was higher than that of the 3D-CRT group, however, there was no statistical difference. The PFS and OS for patients with low-risk groups were better than those of the high-risk group (P<0.05). There was no statistical difference between the M2 group and the M3 group in the 2-year and 5-year OS(P>0.05). There was no statistical difference in the chemotherapy-free group, the EP chemotherapy regimen and the temozolomide (TMZ) chemotherapy group in the 2-year and 5-year PFS and OS(P>0.05). The results of multivariate Cox proportional hazards model analysis showed that the residual, recurrence or metastasis before radiotherapy were independent prognostic factors affecting PFS(P<0.05). Radiotherapy mode was negatively correlated, although there was no statistical significance(P=0.061).Conclusions IMRT can reduce the risk of spinal cord recurrence compared with 3D-CRT, especially the low rate of spinal cord recurrence. The development of the technique of radiotherapy was associated with improvements in medulloblastoma prognosis.

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“…, characterized as WHO group IV, represents the most common malignant pediatric central nervous system (CNS) tumor [1][2][3][4], representing 9.2% of all pediatric brain tumor cases [1,5] and roughly 500 new cases each year in the US. MBs originate in the cerebellum and share molecular signatures with embryonic cerebellar lineages, with metastasis sites commonly include parts of the brain, spinal cord, and, rarely, to extraneural sites [6][7][8].…”

Section: Introductionmentioning

confidence: 99%

In silico analysis of long non-coding RNAs in medulloblastoma and its subgroups

Joshi

Perera

2019

Preprint

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AbstractMedulloblastoma is the most common malignant pediatric brain tumor with high fatality rate. Recent large-scale studies utilizing genome-wide technologies have sub-grouped medulloblastomas into four major subgroups: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4. However, there has yet to be a global analysis of long non-coding RNAs, a crucial part of the regulatory transcriptome, in medulloblastoma. Here, we performed bioinformatic analysis of RNA-seq data from 175 medulloblastoma patients. Differential lncRNA expression sub-grouped medulloblastomas into the four main molecular subgroups. Some of these lncRNAs were subgroup-specific, with a random forest-based machine-learning algorithm identifying an 11-lncRNA diagnostic signature. We also validated the diagnostic signature in patient derived xenograft (PDX) models. We further identified a 17-lncRNA prognostic model using LASSO based penalized Cox’ PH model (Score HR= 13.6301, 95% CI= 8.857-20.98, logrank p-value=< 2e-16). Our analysis represents the first global lncRNA analysis in medulloblastoma. Our results identify putative candidate lncRNAs that could be evaluated for their functional role in medulloblastoma genesis and progression or as diagnostic and prognostic biomarkers.

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Medulloblastoma

Cited by 210 publications

References 114 publications

Splicing Dysregulation as Oncogenic Driver and Passenger Factor in Brain Tumors

Splicing Dysregulation as Oncogenic Driver and Passenger Factor in Brain Tumors

Recurrence pattern and Survival analysis in patients undergoing radiotherapy IMRT technique for medulloblastoma

In silico analysis of long non-coding RNAs in medulloblastoma and its subgroups

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