Search Our Scientific Publications & Authors

Radiother Oncol 2019 Apr 10;136:50-55. Epub 2019 Apr 10.

Department of Pediatric Hematology/Oncology/Hemostaseology, University Medical Center of the Johannes Gutenberg-University Mainz, Germany.

Background And Purpose: While survival times after treatment of medulloblastoma are increasing, little is known about radiochemotherapy (RCT)-induced cerebrovascular changes. High resolution vessel wall imaging (VWI) sequences are an emerging tool for the evaluation of cerebrovascular diseases. We performed VWI in medulloblastoma long-term survivors to screen for late sequelae of RCT. Read More

J Neurosurg Pediatr 2019 Apr 19:1-6. Epub 2019 Apr 19.

Neurogenic stunned myocardium (NSM) is a potentially fatal cause of sudden cardiogenic dysfunction due to an acute neurological event, most commonly aneurysmal subarachnoid hemorrhage in adults. Only two pediatric cases of hydrocephalus-induced NSM have been reported. Here the authors report a third case in a 14-year-old boy who presented with severe headache, decreased level of consciousness, and shock in the context of acute hydrocephalus secondary to fourth ventricular outlet obstruction 3 years after standard-risk medulloblastoma treatment. Read More

Med Dosim 2019 Apr 15. Epub 2019 Apr 15.

Radiation Oncology, Manipal Hospitals, New Delhi, Delhi 110075, India.

Since 2011 when it was first described, the volumetric-modulated arc therapy (VMAT) technique for craniospinal irradiation (CSI) has always seen the use of large arc lengths for the spine fields ranging from 200° to 360°. This study was aimed to do a dosimetric comparison between the large and shorter spinal arc for CSI. For a cohort of 10 patients, 2 VMAT CSI plans were created for each patient, one using the conventional full 360° arc (VMAT_FA) for the spine and the other using 100° posterior arc (VMAT_PA) for 23. Read More

Gene 2019 Apr 13. Epub 2019 Apr 13.

Texas College of Osteopathic Medicine, University of North Texas Health Science Center, Fort Worth, TX 76107, USA; Department of Hematology and Oncology, Cook Children's Medical Center, Fort Worth, TX 76104, USA. Electronic address:

Medulloblastoma (MB) is characterized by highly invasive embryonal neuro-epithelial tumors that metastasize via cerebrospinal fluid. MB is difficult to treat and the chemotherapy is associated with significant toxicities and potential long-term disabilities. Previously, we showed that small molecule, clotam (tolfenamic acid: TA) inhibited MB cell proliferation and tumor growth in mice by targeting, survivin. Read More

J Neurooncol 2019 Apr 13. Epub 2019 Apr 13.

Division of Oncology, Children's Hospital of Philadelphia, 3501 Civic Center Blvd., 10211 CTRB, Philadelphia, PA, 19104, USA.

Purpose: Survivors of pediatric brain tumors often have neurodevelopmental late effects, such as inattention. Symptoms may mirror those of attention-deficit/hyperactivity disorder (ADHD), which affects ~ 5-8% of the general population. This retrospective study of survivors followed at a large tertiary care center examined the prevalence of a clinical diagnosis of ADHD, and risk factors associated with ADHD diagnosis and ADHD-related medication use. Read More

Neuro Oncol 2019 Apr 12. Epub 2019 Apr 12.

Division of Molecular and Developmental Biology, The Institute of Medical Science, The University of Tokyo, Japan.

Background: Embryonal tumors in the central nervous system (CNS) are primary, aggressive, and poorly differentiated pediatric brain tumors. We identified forkhead box R2 (FOXR2) as an oncogene for medulloblastoma through a transposon-based insertional mutagenesis screen. FOXR2 translocation has been identified in a subset of human embryonal tumors of the CNS, designated as CNS neuroblastoma with FOXR2 activation (CNS NB-FOXR2); however, the in vivo functions of FOXR2 remain elusive. Read More

AJNR Am J Neuroradiol 2019 Apr 11. Epub 2019 Apr 11.

Department of Radiation Oncology Tata Memorial Centre Mumbai, India.

J Pediatr Hematol Oncol 2019 Apr 9. Epub 2019 Apr 9.

Departments of Radiation Oncology.

Purpose: The purpose of this work was to study the diversity of sonic hedgehog (SHH) medulloblastoma across different age groups with an emphasis on patterns of relapse.

Methods: All data for the study were obtained through review of medical records, imaging, radiation charts, treatment planning, and chemotherapy details.

Results: Sixty-three patients with SHH medulloblastoma were identified from a prospectively maintained database and classified into 3 groups-infantile: ≤3 years (i-SHH, n=11); pediatric: >3 to <18 years (p-SHH, n=21); and adult: ≥18 years (a-SHH; n=31). Read More

Diagn Pathol 2019 Apr 9;14(1):29. Epub 2019 Apr 9.

Knight Diagnostic Laboratories and Department of Molecular and Medical Genetics, Oregon Health & Science University, Portland, OR, 97239, USA.

Insights into the molecular underpinnings of primary central nervous system tumors have radically changed the approach to tumor diagnosis and classification. Diagnostic emphasis has shifted from the morphology of a tumor under the microscope to an integrated approach based on morphologic and molecular features, including gene mutations, chromosomal copy number alterations, and gene rearrangements. In 2016, the World Health Organization provided guidelines for making an integrated diagnosis that incorporates both morphologic and molecular features in a subset of brain tumors. Read More

Open Biol 2019 Jan;9(1):180145

Department of Molecular Medicine, University of Rome , 'La Sapienza', Rome , Italy.

Cerebellar granule cell progenitors (GCPs) undergo proliferation in the post-natal cerebellum that is dependent on sonic hedgehog (SHH) signalling. Deregulated SHH signalling leads to type 2 medulloblastoma (MB). In this work, a novel cell culture protocol is described, which is suitable for the establishment and long-term maintenance of GCP-derived cells. Read More

Front Neurol 2019 18;10:181. Epub 2019 Mar 18.

Clinical Pathology, IRCCS Regina Elena National Cancer Institute, Rome, Italy.

Cancer patients commonly show abnormal laboratory coagulation tests, indicating a subclinical hypercoagulable condition that contribute to morbidity and mortality. The hypercoagulation status not only increases the risk of thromboembolic events but also influences the tumor biology promoting its growth and progression by stimulating intracellular signaling pathways. Recent molecular studies characterized the role of oncogene and suppressor gene in activating clotting pathways, as an integral feature of the neoplastic transformation. Read More

Sci Rep 2019 Apr 4;9(1):5632. Epub 2019 Apr 4.

Glioma Immunotherapy Group, Division of Neurosurgery, Department of Clinical Sciences, Lund University, Lund, Sweden.

Several chemotherapeutic drugs are now considered to exert anti-tumour effects, by inducing an immune-promoting inflammatory response. Cisplatin is a potent chemotherapeutic agent used in standard medulloblastoma but not glioblastoma protocols. There is no clear explanation for the differences in clinical efficacy of cisplatin between medulloblastomas and glioblastomas, despite the fact that cisplatin is effective in vitro against the latter. Read More

Wiley Interdiscip Rev Dev Biol 2019 Apr 3:e342. Epub 2019 Apr 3.

Key Laboratory of Birth Defects and Related Diseases of Women and Children of Ministry of Education, Sichuan University, Chengdu, China.

Brain tumors such as adult glioblastomas and pediatric high-grade gliomas or medulloblastomas are among the leading causes of cancer-related deaths, exhibiting poor prognoses with little improvement in outcomes in the past several decades. These tumors are heterogeneous and can be initiated from various neural cell types, contributing to therapy resistance. How such heterogeneity arises is linked to the tumor cell of origin and their genetic alterations. Read More

Biosci Rep 2019 Apr 3. Epub 2019 Apr 3.

Capital Medical University, Beijing, China

Long noncoding RNA LOXL1 antisense RNA 1 (lncRNA LOXL1-AS1) was recently found to founction as oncogenic lncRNA in glioblastoma, prostate cancer and medulloblastoma. The role of LOXL1-AS1 in osteosarcoma was still unknown. In our study, we found LOXL1-AS1 expression levels were higher in osteosarcoma tissues and cell lines than normal bone tissues and normal osteoblast cell line, respectively. Read More

Acta Neuropathol Commun 2019 Apr 3;7(1):52. Epub 2019 Apr 3.

Shirsat Laboratory, Advanced Centre for Treatment, Research & Education in Cancer, Tata Memorial Centre, Kharghar, Navi Mumbai, 410210, India.

Genome-wide expression profiling studies have identified four core molecular subgroups of medulloblastoma: WNT, SHH, Group 3 and Group 4. Molecular markers are necessary for accurate risk stratification in the non-WNT subgroups due to the underlying heterogeneity in genetic alterations and overall survival. MiR-204 expression was evaluated in molecularly classified 260 medulloblastomas from an Indian cohort and in 763 medulloblastomas from the MAGIC cohort, SickKids, Canada. Read More

BMC Cancer 2019 Apr 3;19(1):300. Epub 2019 Apr 3.

University of Liverpool, Institute of Integrated Biology, Department of Biochemistry, Centre for Cell Imaging, L69 7ZB, Liverpool, UK.

Background: Solid tumours are less oxygenated than normal tissues. This is called tumour hypoxia and leads to resistance to radiotherapy and chemotherapy. The molecular mechanisms underlying such resistance have been investigated in a range of tumour types, including the adult brain tumours glioblastoma, yet little is known for paediatric brain tumours. Read More

Eur J Med Chem 2019 Jun 26;172:1-15. Epub 2019 Mar 26.

Jiangsu Key Laboratory of Neuropsychiatric Diseases and College of Pharmaceutical Sciences, Soochow University, Suzhou, Jiangsu, 215123, PR China. Electronic address:

The Hedgehog (Hh) pathway plays a critical role during embryonic development by controlling cell patterning, growth and migration. In adults, the function of Hh pathway is curtailed to tissue repair and maintenance. Aberrant reactivation of Hh signaling has been linked to tumorigenesis in various cancers, such as basal cell carcinoma (BCC) and medulloblastoma. Read More

Clin Transl Oncol 2019 Apr 1. Epub 2019 Apr 1.

Pediatric Hematology, Oncology and Stem Cell Department, Hospital Infantil Universitario Niño Jesús, Av. Menéndez Pelayo, 65, 28009, Madrid, Spain.

Purpose: Elevated mortality and morbidity rates persist in pediatric patients with medulloblastoma. We present a clinical audit of a real-world cohort of patients in search for pragmatic measures to improve their management and outcome.

Methods/patients: All pediatric patients with medulloblastoma treated between 2003 and 2016 at a Spanish reference center were reviewed. Read More

Eur J Hum Genet 2019 Apr 1. Epub 2019 Apr 1.

Department of Neurosurgery, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini, 5, 16147, Genoa, Italy.

De novo DDX3X variants account for 1-3% of syndromic intellectual disability (ID) in females and have been occasionally reported in males. Furthermore, somatic DDX3X variants occur in several aggressive cancers, including medulloblastoma. We report three unrelated females with severe ID, dysmorphic features, and a common brain malformative pattern characterized by malformations of cortical development, callosal dysgenesis, basal ganglia anomalies, and midbrain-hindbrain malformations. Read More

Sci Rep 2019 Mar 29;9(1):5309. Epub 2019 Mar 29.

Department of Biomedical Engineering, Pratt School of Engineering, Duke University, 101 Science Drive, Durham, NC, 27705, USA.

Treatment of neuroepithelial cancers remains a daunting clinical challenge, particularly due to an inability to address rampant invasion deep into eloquent regions of the brain. Given the lack of access, and the dispersed nature of brain tumor cells, we explore the possibility of electric fields inducing directed tumor cell migration. In this study we investigate the properties of populations of brain cancer undergoing electrotaxis, a phenomenon whereby cells are directed to migrate under control of an electrical field. Read More

Front Pediatr 2019 14;7:67. Epub 2019 Mar 14.

Institute of Molecular Biology and Pathology, National Research Council, Rome, Italy.

Central Nervous System tumors are the leading cause of cancer-related death in children, and medulloblastoma has the highest incidence rate. The current therapies achieve a 5-year survival rate of 50-80%, but often inflict severe secondary effects demanding the urgent development of novel, effective, and less toxic therapeutic strategies. Historically identified on a histopathological basis, medulloblastoma was later classified into four major subgroups-namely WNT, SHH, Group 3, and Group 4-each characterized by distinct transcriptional profiles, copy-number aberrations, somatic mutations, and clinical outcomes. Read More

Cancer Gene Ther 2019 Mar 28. Epub 2019 Mar 28.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, 35233, USA.

Primary malignant central nervous system (CNS) tumors are the leading cause of childhood cancer-related death and morbidity. While advances in surgery, radiation, and chemotherapy have improved the survival rates in children with malignant brain tumors, mortality persists in certain subpopulations and current therapies are associated with extreme morbidity. This is especially true for children with malignant infratentorial tumors. Read More

World J Surg Oncol 2019 Mar 27;17(1):59. Epub 2019 Mar 27.

Neuro-oncology Clinic, National Cancer Center, Goyang, Republic of Korea.

Background: Leptomeningeal carcinomatosis (LMC) is frequently associated with hydrocephalus, which quickly devastates the performance of the patient. Cerebrospinal fluid (CSF) shunt is a widely accepted treatment of choice, but the clinical outcomes in patients with LMC are not well studied. This study aimed to examine the efficacy of a CSF shunt in patients with LMC. Read More

Future Med Chem 2019 Mar 26;11(6):617-638. Epub 2019 Mar 26.

Departamento de Química Orgánica, Facultad de Química y de Farmacia, Pontificia Universidad Católica de Chile, Santiago de Chile 702843, Chile.

Since the Hedgehog signaling pathway has been associated with cancer, it has emerged as a therapeutic target for cancer therapy. The main target among the key Hedgehog proteins is the GPCR-like Smo receptor. Therefore, some Smo antagonists that have entered clinical trials, including the US FDA-approved drugs vismodegib and sonidegib, to treat basal cell carcinoma and medulloblastoma. Read More

J Cell Physiol 2019 Mar 25. Epub 2019 Mar 25.

Department of Psychology, Division of Neuroscience and "Daniel Bovet" Neurobiology Research Center, Sapienza University of Rome, Rome, Italy.

Proteins belonging to the TGFβ-stimulated clone 22 domain (TSC22D) family display a repertoire of activities, regulating cell proliferation and differentiation. The tumor suppressor activity of the first identified member of the family, TSC22D1 (formerly named TSC-22), has been extensively studied, but afterward a longer isoform encoded by the same gene turned out to play an opposite role. We have previously characterized the role of TSC22D1 and TSC22D4 in cell differentiation using granule neurons (GNs) isolated from the mouse cerebellum. Read More

J Clin Neurosci 2019 Mar 21. Epub 2019 Mar 21.

Neurological Surgery Department, Hospital General Universitario Gregorio Marañón, C/ Doctor Esquerdo, 46 28007 Madrid, Spain.

Background: To describe an exceptional case of late recurrence of medulloblastoma after 17 years of complete remission.

Case Description: A 42-year-old male consulted in ER for 10-day occipital headache. He had a previous history of cerebellar medulloblastoma 17 years ago treated with gross total resection, chemotherapy and radiotherapy. Read More

Brain Stimul 2019 Mar 12. Epub 2019 Mar 12.

Keystone Rural Health Center, Keystone Behavioral Health, 110 Chambers Hill Dr, Chambersburg, PA, 17201, United States.

Neurochirurgie 2019 Mar 20. Epub 2019 Mar 20.

Équipe "Signalisation, développement et tumeurs cérébrales", unité Inserm U1021, Institut Curie, université Paris-Saclay, 15, rue Georges-Clémenceau, Orsay, France.

Introduction: Medulloblastoma is the most common type of pediatric malignant brain tumor where the most important amount of clinical and radiological data has been accumulated in recent years. This has led to its sophistication in the management of these patients with a clear benefit for the patients. Long-term outcome and sequelae have been described and their causes well understood such as preventive measures which can now be implemented. Read More

Childs Nerv Syst 2019 Mar 21. Epub 2019 Mar 21.

Department of Radiology Emergency, "Sapienza" University, Rome, Italy.

Aim: The aim of the study is to assess tumor response, treatment-related toxicities, progression-free survival (PFS), and overall survival (OS) in patients with relapsed/refractory brain tumors treated with bevacizumab-containing regimen.

Methods: Patients that had received I and II line treatments with or without megatherapy were included. Doses and schedule were as follows: bevacizumab (BVZ) 10 mg/kg i. Read More

Redox Biol 2019 Mar 13;24:101163. Epub 2019 Mar 13.

Department of Genetics, Faculty of Biotechnology, University of Rzeszow, Pigonia 1, 35-310 Rzeszow, Poland. Electronic address:

Medulloblastoma (MB) is a common and highly aggressive pediatric brain tumor of a heterogeneous nature. According to transcriptome-based profiling, four molecular subgroups of MB have been revealed, namely WNT, SHH, Group 3 and Group 4. High MYC mRNA expression and MYC gene amplification in MB have been considered as indicators of poor prognosis. Read More

Cancer Res 2019 Mar 19. Epub 2019 Mar 19.

Medical Biophysics, University of Toronto

Medulloblastoma (MB) is a pediatric malignant brain tumor comprised of four different subgroups (WNT, SHH, Group 3, Group 4), each of which are a unique biological entity with distinct clinico-pathological, molecular, and prognostic characteristics. While risk stratification of MB patients based on molecular features may offer personalized therapies, conventional subgroup identification methods take too long and are unable to deliver subgroup information intraoperatively. This limitation prevents subgroup-specific adjustment of the extent or the aggressiveness of the tumor resection by the neurosurgeon. Read More

J Hematol Oncol 2019 Mar 15;12(1):29. Epub 2019 Mar 15.

2nd Department of Pediatrics, Semmelweis University, Tűzoltó u. 7-9, Budapest, H-1094, Hungary.

Childhood medulloblastomas (MB) are heterogeneous and are divided into four molecular subgroups. The provisional non-wingless-activated (WNT)/non-sonic hedgehog-activated (SHH) category combining group 3 and group 4 represents over two thirds of all MBs, coupled with the highest rates of metastases and least understood pathology. The molecular era expanded our knowledge about molecular aberrations involved in MB tumorigenesis, and here, we review processes leading to non-WNT/non-SHH MB formations. Read More

Cancer Res 2019 Apr 12;79(8):1967-1980. Epub 2019 Mar 12.

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

A subset of group 3 medulloblastoma frequently harbors amplification or overexpression of lacking additional focal aberrations, yet it remains unclear whether overexpression alone can induce tumorigenesis and which cells give rise to these tumors. Here, we showed that astrocyte progenitors in the early postnatal cerebellum were susceptible to transformation by . The resulting tumors specifically resembled human group 3 medulloblastoma based on histology and gene-expression profiling. Read More

Acta Neuropathol 2019 Apr 8;137(4):675-678. Epub 2019 Mar 8.

Pediatric Oncology Department, SIREDO Oncology Centre (Care, Innovation, Research in Pediatric, Adolescent and Young Adults Oncology), Institut Curie, 26, rue d'Ulm, 75248, Paris Cedex 05, France.

Pediatr Blood Cancer 2019 Jun 8;66(6):e27694. Epub 2019 Mar 8.

Programme in Developmental and Stem Cell Biology, Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada.

Most medulloblastoma protocols worldwide include vincristine during radiation and chemotherapy. A significant dose-limiting toxicity is peripheral neuropathy; however, there is a paucity of data to support the view that omission of vincristine does not impact survival. Herein we report two adolescent patients with Group 4 and SHH medulloblastoma, where vinblastine successfully replaced vincristine with resolution of their peripheral neuropathy. Read More

Support Care Cancer 2019 Mar 8. Epub 2019 Mar 8.

Colorado School of Public Health, Aurora, USA.

Background: Pediatric brain tumor survivors (PBTSs) are at risk of impairments in social competence. Limited information is available regarding nominations and reciprocated nominations of PBTSs as best friends and factors which may predict these.

Procedure: Caregivers of children (n = 32) aged 8-16 (38% low-grade glioma, 34% medulloblastoma, 28% other) completed ratings of child adjustment at baseline (T1) and PBTSs and classmates completed the Three Best Friends measure approximately 12 months later (T2). Read More

Front Pharmacol 2019 21;10:89. Epub 2019 Feb 21.

Shanghai Engineering Research Center of Molecular Therapeutics and New Drug Development, School of Chemistry and Molecular Engineering, East China Normal University, Shanghai, China.

Inhibition of aberrant Hedgehog (Hh) pathway had been proved to be a promising therapeutic intervention in cancers like basal cell carcinoma (BCC), medulloblastoma (MB), and so on. Two drugs (Vismodegib, Sonidegib) were approved to treat BCC and more inhibitors are in clinical investigation. However, the adverse effects and drug resistance restricted the use of Hh inhibitors. Read More

Mol Cancer Res 2019 Mar 6. Epub 2019 Mar 6.

Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois.

Medulloblastoma is a highly malignant pediatric brain tumor associated with poor outcome. Developing treatments that target the cancer stem cell (CSC) population in medulloblastoma are important to prevent tumor relapse and induce long-lasting clinical responses. We utilized medulloblastoma neurospheres that display CSC characteristics and found activation of the PI3K/AKT pathway in sphere-forming cells. Read More

Cells 2019 Mar 5;8(3). Epub 2019 Mar 5.

Centre of New Technologies, University of Warsaw, Banacha 2c, 02-097 Warsaw, Poland.

Medulloblastoma is a brain tumor that arises predominantly in infants and children. It is the most common pediatric brain malignancy. Around 25% of medulloblastomas are driven by constitutive activation of the Hedgehog signaling pathway. Read More

J Chin Med Assoc 2019 Feb;82(2):148-154

Faculty of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC.

Background: Despite aggressive treatment including surgery, radiotherapy, and adjuvant chemotherapy, the outcome of pediatric high-risk embryonal brain tumors remains poor; especially in young children, in whom early radiotherapy inevitably brings significant long-term morbidities. Single or tandem autologous stem cell transplant has been reported to improve outcomes; but optimal use is not well defined.

Methods: Pediatric patients with high-risk embryonal brain tumors who underwent tandem transplant as consolidation from August 2011 to December 2017 were included. Read More

Scand J Work Environ Health 2019 Mar 6. Epub 2019 Mar 6.

The Danish Cancer Society Research Center, Strandboulevarden 49, DK-2100 Copenhagen Ø, Denmark.

Objectives Parental exposures and offspring's risk of cancer have been studied with inconsistent results. We investigated parental employment in painting and printing industries and risk of childhood leukemia, central nervous system (CNS) cancers, and prenatal cancers (acute lymphoblastic leukemia, Wilms tumor, medulloblastoma, neuroblastoma, retinoblastoma, and hepatoblastoma). Methods Using Danish registries, children aged ≤19 years diagnosed from 1968-2015 with leukemia (N=1999), CNS cancers (N=1111) or prenatal cancers (N=2704) were linked to parents and their employment history one year before birth to birth for fathers, and one year before birth to one year after for mothers. Read More

Front Mol Biosci 2019 19;6. Epub 2019 Feb 19.

Department of Pharmacology, University of California Davis School of Medicine, Davis, CA, United States.

The transcription factor MXD3 is an atypical member of the MYC/MAX/MXD transcriptional network and has been previously shown to be an important regulator of cell proliferation. MXD3 has been shown to be overexpressed and to be required for medulloblastoma and acute lymphoblastic leukemia cell proliferation. In this study we leveraged datasets from The Cancer Genome Atlas to examine MXD3 across several cancers. Read More

J Neurosurg Pediatr 2019 Mar;23(3):261-273

Brain tumors are the most common solid tumors in children, and, unfortunately, many subtypes continue to have a suboptimal long-term outcome. During the last several years, however, remarkable advances in our understanding of the molecular underpinnings of these tumors have occurred as a result of high-resolution genomic, epigenetic, and transcriptomic profiling, which have provided insights for improved tumor categorization and molecularly directed therapies. While tumors such as medulloblastomas have been historically grouped into standard- and high-risk categories, it is now recognized that these tumors encompass four or more molecular subsets with distinct clinical and molecular characteristics. Read More

Acta Neuropathol Commun 2019 Mar 4;7(1):33. Epub 2019 Mar 4.

Department of Pediatrics Ribeirão Preto Medical School, Hospital das Clínicas, University of São Paulo, Av. Bandeirantes 3900, Ribeirão Preto, São Paulo, Brazil.

Next-generation sequencing platforms are routinely used for molecular assignment due to their high impact for risk stratification and prognosis in medulloblastomas. Yet, low and middle-income countries still lack an accurate cost-effective platform to perform this allocation. TaqMan Low Density array (TLDA) assay was performed using a set of 20 genes in 92 medulloblastoma samples. Read More

Cancer Epidemiol 2019 Apr 1;59:208-214. Epub 2019 Mar 1.

Cancer Council Queensland, Brisbane, Australia; Menzies Health Institute Queensland, Griffith University, Gold Coast, Australia; Institute for Resilient Regions, University of Southern Queensland, Brisbane, Australia; School of Public Health, University of Queensland, Brisbane, Australia. Electronic address:

Background: Stage of cancer at diagnosis is one of the strongest predictors of survival and is essential for population cancer surveillance, comparison of cancer outcomes and to guide national cancer control strategies. Our aim was to describe, for the first time, the distribution of cases by stage at diagnosis and differences in stage-specific survival on a population basis for a range of childhood solid cancers in Australia.

Methods: The study cohort was drawn from the population-based Australian Childhood Cancer Registry and comprised children (<15 years) diagnosed with one of 12 solid malignancies between 2006 and 2014. Read More

J Nat Prod 2019 Mar 4. Epub 2019 Mar 4.

Natural Products Branch, Developmental Therapeutics Program, Division of Cancer Treatment and Diagnosis , National Cancer Institute , Frederick , Maryland 21702 , United States.

An extract of the plant Anacolosa clarkii was obtained from the NCI Natural Products Repository, and it showed cytotoxic activity toward several types of pediatric solid tumor cell lines. Bioassay-guided fractionation led to the purification of eight new clerodane diterpenes [anacolosins A-F (1-6) and corymbulosins X and Y (7 and 8)] and two known compounds (9 and 10) that contained an isozuelanin skeleton. The structures of the new natural products were determined using 1D and 2D NMR and HRESIMS data, while the relative and absolute configurations of the compounds were assessed using a combination of H NMR coupling constant data, ROESY experiments, ECD (electronic circular dichroism) and VCD (vibrational circular dichroism) spectroscopy, chemical methods (including Mosher and 2-naphthacyl esterification), and chiral HPLC analyses. Read More

J Neurooncol 2019 May 4;142(3):435-444. Epub 2019 Mar 4.

Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy.

Purpose: Elongation of telomeres is necessary for tumor cell immortalization and senescence escape; neoplastic cells use to alternative pathways to elongate telomeres: telomerase reactivation or a telomerase-independent mechanism termed alternative lengthening of telomeres (ALT). Telomerase and ALT pathway has been explored in adult and pediatric gliomas and medulloblastomas (MDBs); however, these mechanisms were not previously investigated in MDBs metastatic at the onset. Therefore, we analyzed the activation of telomerase and ALT pathway in a homogenous cohort of 43 pediatric metastatic medulloblastomas, to investigate whether telomere elongation could play a role in the biology of metastatic MDB. Read More

Acta Neuropathol 2019 Apr 4;137(4):657-673. Epub 2019 Mar 4.

Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

The TCF4 gene encodes for the basic helix-loop-helix transcription factor 4 (TCF4), which plays an important role in the development of the central nervous system (CNS). Haploinsufficiency of TCF4 was found to cause Pitt-Hopkins syndrome (PTHS), a severe neurodevelopmental disorder. Recently, the screening of a large cohort of medulloblastoma (MB), a highly aggressive embryonal brain tumor, revealed almost 20% of adult patients with MB of the Sonic hedgehog (SHH) subtype carrying somatic TCF4 mutations. Read More

Indian J Cancer 2018 Oct-Dec;55(4):372-376

Department of Radiotherapy and Nuclear Medicine, National Cancer Institute, Cairo University, Giza; Department of Radiotherapy, Children Cancer Hospital, Cairo, Egypt.

Background: Craniospinal irradiation (CSI) is the standard radiation therapy treatment for medulloblastoma. The aim of this study was to estimate and compare the lifetime risk of radiation-induced secondary cancer in pediatric medulloblastoma patients using three-dimensional conformal radiotherapy (3D-CRT) and intensity-modulated radiotherapy (IMRT).

Materials And Methods: 3D-CRT and IMRT plans were performed for 10 CSI pediatric patients. Read More

Acta Neuropathol 2019 Mar 2. Epub 2019 Mar 2.

Clinical Cooperation Unit Neuropathology (B300), German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidelberg, Germany.

Desmoplastic/nodular medulloblastomas (DNMB) and medulloblastomas with extensive nodularity (MBEN) were outlined in the current WHO classification of tumors of the nervous system as two distinct histological MB variants. However, they are often considered as cognate SHH MB entities, and it is a reason why some clinical MB trials do not separate the patients with DNMB or MBEN histology. In the current study, we performed an integrated DNA/RNA-based molecular analysis of 83 DNMB and 36 MBEN to assess the etiopathogenetic relationship between these SHH MB variants. Read More