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Ochsner J 2022 ;22(2):176-181

Division of Neurosurgery, SBH Health System, Bronx, NY.

Medulloblastoma of the posterior fossa is commonly encountered in pediatric populations but rarely reported in adults. Adult cases of medulloblastoma typically occur in younger patients, tend to arise intra-axially within the cerebellar hemisphere, and usually exhibit classic histopathologic features. A 54-year-old male presented with headaches, dizziness, gait instability, and frequent falls that had worsened during the prior 3 months. Read More

Front Oncol 2022 7;12:903830. Epub 2022 Jun 7.

Department of Pediatrics, Emory University, Atlanta, GA, United States.

Medulloblastoma (MB) is the most common malignant brain tumor in children with standard of care consisting of surgery, radiation, and chemotherapy. Recent molecular profiling led to the identification of four molecularly distinct MB subgroups - Wingless (WNT), Sonic Hedgehog (SHH), Group 3, and Group 4. Despite genomic MB characterization and subsequent tumor stratification, clinical treatment paradigms are still largely driven by histology, degree of surgical resection, and presence or absence of metastasis rather than molecular profile. Read More

Pharmaceuticals (Basel) 2022 May 26;15(6). Epub 2022 May 26.

Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA.

Pediatric brain tumors have surpassed leukemia as the leading cause of cancer-related death in children. Several landmark studies from the last two decades have shown that many pediatric brain tumors are driven by epigenetic dysregulation within specific developmental contexts. One of the major determinants of epigenetic control is the histone code, which is orchestrated by a number of enzymes categorized as writers, erasers, and readers. Read More

Int J Environ Res Public Health 2022 Jun 9;19(12). Epub 2022 Jun 9.

Physical and Rehabilitation Medicine, Ghent University Hospital, 9000 Ghent, Belgium.

Background: Short- and long-term consequences after treatment for childhood fossa posterior tumors are extensively reported in the literature; however, papers highlighting physical function throughout rehabilitation and its correlation with Intelligence Quotient (IQ) are sparse. This study aims to describe the physical functioning and IQ of these survivors, their progression during rehabilitation, and the association with histopathological tumor classification. Additionally, the correlation between gross motor functioning and cognitive functioning was investigated. Read More

Clin Cancer Res 2022 Jun 23:OF1-OF14. Epub 2022 Jun 23.

Center for Cancer and Immunology, Brain Tumor Institute, Children's National Health System, Washington, District of Columbia.

Purpose: Patients with MYC-amplified medulloblastoma (MB) have poor prognosis and frequently develop recurrence, thus new therapeutic approaches to prevent recurrence are needed.

Experimental Design: We evaluated OLIG2 expression in a panel of mouse Myc-driven MB tumors, patient MB samples, and patient-derived xenograft (PDX) tumors and analyzed radiation sensitivity in OLIG2-high and OLIG2-low tumors in PDX lines. We assessed the effect of inhibition of OLIG2 by OLIG2-CRISPR or the small molecule inhibitor CT-179 combined with radiotherapy on tumor progression in PDX models. Read More

Cancer Res 2022 Jun 22. Epub 2022 Jun 22.

The University of Texas Southwestern Medical Center, Dallas, TX, United States.

Intratumor epigenetic heterogeneity is emerging as a key mechanism underlying tumor evolution and drug resistance. Epigenetic abnormalities frequently occur in medulloblastoma, the most common childhood malignant brain tumor. Medulloblastoma is classified into four subtypes including SHH medulloblastoma, which is characterized by elevated SHH signaling and a cerebellum granule neuron precursor (CGNP) cell-of-origin. Read More

J Clin Neurosci 2022 Jun 18;102:65-70. Epub 2022 Jun 18.

Olivia Newton John Cancer Research Institute, Melbourne, Australia; Austin Health, Melbourne, Australia; La Trobe University, School of Cancer Medicine, Heidelberg, Victoria, Australia; Department of Medical Biology, The University of Melbourne, Melbourne, Australia.

Medulloblastoma in adult patients is a rare condition with limited contemporary demographic and treatment outcome data available in an Australian population. We conducted a retrospective review of patterns of care and outcomes of adult patients diagnosed with medulloblastoma treated at major neuro-oncology centres across Australia between January 2010 and December 2019. A total of 80 patients were identified and the median follow-up after diagnosis was 59. Read More

J Neuroinflammation 2022 Jun 20;19(1):159. Epub 2022 Jun 20.

Laboratory of Molecular Neuropathology, Pediatric Cancer Center, College of Pharmaceutical Sciences, Soochow University, Suzhou, China.

Background: Medulloblastoma (MB) is the most common malignant brain tumor in children. Approximately one-third of MB patients remain incurable. Understanding the molecular mechanism of MB tumorigenesis is, therefore, critical for developing specific and effective treatment strategies. Read More

Front Public Health 2022 2;10:914536. Epub 2022 Jun 2.

Department of Integrated Care Management Center, West China Hospital, Sichuan University, Chengdu, China.

Background: is the most common malignant brain tumor of childhood, accounting for 6 to 7 percent of all childhood CNS tumors. The purpose of this study was to evaluate the economic efficacy of a bevacizumab combined with + irinotecan regimen for the treatment of recurrent pediatric in China.

Methods: The data analyzed were from a randomized phase II screening trial that showed an improved survival benefit in child patients with recurrent treated with a T+I+B combination regimen. Read More

Int J Radiat Oncol Biol Phys 2022 Jun 15. Epub 2022 Jun 15.

Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address:

Background: For children, craniospinal irradiation (CSI) with photons is associated with significant toxicities. The use of electrons for spinal fields is hypothesized to spare anterior structures but the long-term effects remain uncertain. We studied late effects of CSI using electrons for spinal radiotherapy (RT). Read More

Neuromolecular Med 2022 Jun 18. Epub 2022 Jun 18.

Cancer and Neurobiology Laboratory, Experimental Research Center, Clinical Hospital (CPE-HCPA), Federal University of Rio Grande do Sul, Porto Alegre, RS, 90035-003, Brazil.

Medulloblastoma (MB) is a malignant brain tumor that afflicts mostly children and adolescents and presents four distinct molecular subgroups, known as WNT, SHH, Group 3, and Group 4. ZEB1 is a transcription factor that promotes the expression of mesenchymal markers while restraining expression of epithelial and polarity genes. Because of ZEB1 involvement in cerebellum development, here we investigated the role of ZEB1 in MB. Read More

Mol Biol Rep 2022 Jun 18. Epub 2022 Jun 18.

Neuroscience Research Center, Faculty of Medical Sciences, Lebanese University, Hadath, Beirut, Lebanon.

Medulloblastoma is the most common malignant brain tumor of childhood accounting for about 60% of all pediatric embryonal tumors. Despite improvements in the overall survival rate, this tumor still lacks an efficient, reliable, and less toxic therapeutic approach. Characterization of the molecular mechanisms involved in medulloblastoma initiation and progression is a crucial step for the development of effective therapies. Read More

Pediatr Blood Cancer 2022 Jun 17:e29847. Epub 2022 Jun 17.

Bristol Haematology & Oncology Centre, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK.

Background: Pituitary insufficiency is a common toxicity of cranial radiotherapy received in childhood for central nervous system, head and neck and haematological malignancies. There is a recognised deficiency pattern and correlation with prescribed radiotherapy dose, however correlation with measured pituitary dose (which can be minimised with modern radiotherapy techniques) has not previously been assessed.

Procedure: Retrospective analysis of measured pituitary dose and endocrine outcomes of patients receiving cranial, total body or head and neck photon beam radiotherapy at a tertiary centre July 2008 - October 2019. Read More

J Immunother Cancer 2022 Jun;10(6)

Melanoma, Cancer Immunotherapy and Innovative Therapy, Istituto Nazionale Tumori IRCCS Fondazione G. Pascale, Napoli, Italy

The Sonic hedgehog (Shh) signaling pathway is an essential pathway in the human body that plays an important role in embryogenesis and tissue homeostasis. Aberrant activation of this pathway has been linked to the development of different diseases, ranging from cancer to immune dysregulation and infections.Uncontrolled activation of the pathway through sporadic mutations or other mechanisms is associated with cancer development and progression in various malignancies, such as basal cell carcinoma, medulloblastoma, pancreatic cancer, breast cancer and small-cell lung carcinoma. Read More

Mol Cancer Ther 2022 Jun 16. Epub 2022 Jun 16.

St. Jude Children's Research Hospital, Memphis, TN, United States.

Group3 (G3) medulloblastoma (MB) is one of the deadliest forms of the disease for which novel treatment is desperately needed. Here we evaluate ribociclib, a highly selective CDK4/6 inhibitor, with gemcitabine in mouse and human G3MBs. Ribociclib central nervous system (CNS) penetration was assessed by in vivo microdialysis and by immunohistochemistry and gene expression studies and found to be CNS-penetrant. Read More

J Control Release 2022 Jun 17;348:553-571. Epub 2022 Jun 17.

Department of Pharmaceutical Technology and Chemistry, School of Pharmacy and Nutrition, Universidad de Navarra, C/Irunlarrea 1, 31008 Pamplona, Spain; Instituto de Investigación Sanitaria de Navarra, IdiSNA, C/Irunlarrea 3, 31008 Pamplona, Spain. Electronic address:

Embryonal tumors of the nervous system are neoplasms predominantly affecting the pediatric population. Among the most common and aggressive ones are neuroblastoma (NB) and medulloblastoma (MB). NB is a sympathetic nervous system tumor, which is the most frequent extracranial solid pediatric cancer, usually detected in children under two. Read More

J Pediatr Hematol Oncol 2022 Mar 25. Epub 2022 Mar 25.

Service of Hematology/Oncology, Hospital JP Garrahan.

Medulloblastoma has a reduced incidence in Down syndrome (DS). This protective characteristic has not been clarified yet. Here, we report the second case of SHH medulloblastoma and DS documented in the literature. Read More

Pediatr Blood Cancer 2022 Jun 10:e29838. Epub 2022 Jun 10.

Division of Pediatric Hematology/Oncology and Bone Marrow Transplantation, The Hospital for Sick Children, Toronto, Ontario, Canada.

Therapeutic strategies avoiding craniospinal irradiation were developed for young children with medulloblastoma to improve survival while protecting the neurocognitive outcomes of these vulnerable patients. These strategies most commonly rely on high-dose chemotherapy with stem cell rescue or conventional chemotherapy combined with intraventricular chemotherapy or conventional chemotherapy with adjuvant focal irradiation. Over the past decade, our growing understanding of the molecular landscape of medulloblastoma has transformed how we risk stratify and allocate treatment in this young age group. Read More

Cancers (Basel) 2022 Jun 1;14(11). Epub 2022 Jun 1.

Proton Therapy Unit, Santa Chiara Hospital, Azienda Provinciale per i Servizi Sanitari (APSS), 38123 Trento, Italy.

Medulloblastoma is the most common malignant brain tumor in children. Even if current treatment dramatically improves the prognosis, survivors often develop long-term treatment-related sequelae. The current radiotherapy standard for medulloblastoma is craniospinal irradiation with a boost to the primary tumor site and to any metastatic sites. Read More

Cancers (Basel) 2022 May 28;14(11). Epub 2022 May 28.

Department of Neurosurgery, University Hospital of Basel, 4031 Basel, Switzerland.

Background: Pediatric brain tumors are the most common solid tumor in children. Traditionally, tumor diagnosis and molecular analysis were carried out on tumor tissue harvested either via biopsy or resection. However, liquid biopsy allows analysis of circulating tumor DNA in corporeal fluids such as cerebrospinal fluid or blood. Read More

Cells 2022 06 2;11(11). Epub 2022 Jun 2.

Division of Translational Oncology, Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA.

Here, we report that Dino, a lncRNA required for p53 signaling, suppresses spontaneous tumorigenesis in mice. mice develop significantly more malignant tumors than littermate controls, consisting predominantly of sarcomas, B cell lymphomas and additional rare tumors. While the prevalence of lymphomas and sarcomas in mice is similar to that of mice with p53 loss, important distinctions emerged. Read More

Commun Biol 2022 Jun 9;5(1):569. Epub 2022 Jun 9.

Donald K. Johnson Eye Institute, Krembil Research Institute, University Health Network, Toronto, ON, M5T 2S8, Canada.

Spontaneous mouse models of medulloblastoma (MB) offer a tractable system to study malignant progression in the brain. Mouse Sonic Hedgehog (Shh)-MB tumours first appear at postnatal stages as preneoplastic changes on the surface of the cerebellum, the external granule layer (EGL). Here we compared traditional histology and 3DISCO tissue clearing in combination with light sheet fluorescence microscopy (LSFM) to identify and quantify preneoplastic changes induced by disrupting stromal Norrin/Frizzled 4 (Fzd4) signalling, a potent tumour inhibitory signal in two mouse models of spontaneous Shh-MB. Read More

Genes Dev 2022 May;36(9-10):514-532

Tumor Initiation and Maintenance Program, National Cancer Institute-Designated Cancer Center, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, California 92037, USA.

Medulloblastoma is an aggressive brain tumor that occurs predominantly in children. Despite intensive therapy, many patients die of the disease, and novel therapies are desperately needed. Although immunotherapy has shown promise in many cancers, the low mutational burden, limited infiltration of immune effector cells, and immune-suppressive microenvironment of medulloblastoma have led to the assumption that it is unlikely to respond to immunotherapy. Read More

Clin Neuroradiol 2022 Jun 8. Epub 2022 Jun 8.

Department of Radiology, Division of Neuroradiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Purpose: This study aimed to evaluate the application of apparent diffusion coefficient (ADC) histogram analysis to differentiate posterior fossa tumors (PFTs) in children.

Methods: A total of 175 pediatric patients with PFT, including 75 pilocytic astrocytomas (PA), 59 medulloblastomas, 16 ependymomas, and 13 atypical teratoid rhabdoid tumors (ATRT), were analyzed. Tumors were visually assessed using DWI trace and conventional MRI images and manually segmented and post-processed using parametric software (pMRI). Read More

Eur J Cancer 2022 Jul 4;170:196-208. Epub 2022 Jun 4.

Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; Department of Pharmaceutical Sciences, University Utrecht, Utrecht, Netherlands. Electronic address:

Background: Childhood cancer is still a leading cause of death around the world. To improve outcomes, there is an urgent need for tailored treatment. The systematic evaluation of existing preclinical data can provide an overview of what is known and identify gaps in the current knowledge. Read More

Neuro Oncol 2022 Jun 7. Epub 2022 Jun 7.

Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA.

Background: Medulloblastoma (MB) is a rare central nervous system malignancy of adults, with limited contemporary studies to define treatment guidelines and expected late toxicity.

Methods: A single-center, retrospective study was conducted of patients age ≥18-years from 1997-2019 with MB and who were treated with postoperative radiotherapy. Late toxicity was defined as a minimum of 18-months from diagnosis. Read More

Neurooncol Adv 2022 Jan-Dec;4(1):vdac016. Epub 2022 Feb 9.

Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.

Background: Medulloblastoma is a rare diagnosis among adolescents and young adults (AYA). Though prognostic factors and treatment are well characterized among children with medulloblastoma, equivalent data for AYA are sparse. We conducted a systematic review to identify predictors of survival among AYA with medulloblastoma. Read More

Neurochirurgie 2022 Jun 3. Epub 2022 Jun 3.

Institute of Cancer & Genomic Science, University of Birmingham, B152TT, Birmingham, United Kingdom.

Background: Differential diagnosis between medulloblastoma (MB), ependymoma (EP) and astrocytoma (PA) is important due to differing medical treatment strategies and predicted survival. The aim of this study was to investigate non-invasive MRI-based radiomic analysis of whole tumors to classify the histologic tumor types of pediatric posterior fossa brain tumor and improve the accuracy of discrimination, using a random forest classifier.

Methods: MRI images of 99 patients, with 59 MBs, 13 EPs and 27 PAs histologically confirmed by surgery and pathology before treatment, were included in this retrospective study. Read More

Neurooncol Adv 2022 Jan-Dec;4(1):vdac062. Epub 2022 Apr 22.

Department of Neurology/Brain Tumor Center, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.

Background: Ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors occur relative frequently in children, but are rare central nervous system (CNS) tumors in adults. In this population-based survey, we established incidence, treatment, and survival patterns for these tumors diagnosed in adult patients (≥18 years) over a 30-year period (1989-2018).

Methods: Data on 1384 ependymomas, 454 pilocytic astrocytomas, 205 medulloblastomas, and 112 intracranial germ cell tumors were obtained from the Netherlands Cancer Registry (NCR) on the basis of a histopathological diagnosis. Read More

Eur J Cancer 2022 Jul 31;170:179-193. Epub 2022 May 31.

Institut d'Hématologie et d'Oncologie Pédiatrique (iHOPe), Centre Léon Bérard, Lyon, France; Département d'Innovation Thérapeutique et d'Essais Précoces (DITEP), Gustave Roussy, Villejuif, France; Laboratoire de Recherche Translationelle en Immunothérapies, INSERM U1015, Gustave Roussy, Villejuif, France; Centre d'Investigation Clinique BIOTHERIS, INSERM CIC1428, Gustave Roussy, Villejuif, France; Faculté de Médecine, Université Paris Saclay, Le Kremlin-Bicetre, France. Electronic address:

Background: The clinical development of immune checkpoint-targeted immunotherapies has been disappointing so far in paediatric solid tumours. However, as opposed to adults, very little is known about the immune contexture of paediatric malignancies.

Methods: We investigated by gene expression and immunohistochemistry (IHC) the immune microenvironment of five major paediatric cancers: Ewing sarcoma (ES), osteosarcoma (OS), rhabdomyosarcoma (RMS), medulloblastoma (MB) and neuroblastoma (NB; 20 cases each; n = 100 samples total), and correlated them with overall survival. Read More