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12 pages, 1721 KiB

Open AccessArticle

Posterior Fossa Tumours in the First Year of Life: A Two-Centre Retrospective Study

by Stefania Picariello, Pietro Spennato, Jonathan Roth, Nir Shimony, Alessandra Marini, Lucia De Martino, Giancarlo Nicosia, Giuseppe Mirone, Maria Serena De Santi, Fabio Savoia, Maria Elena Errico, Lucia Quaglietta, Shlomi Costantini and Giuseppe Cinalli

Diagnostics 2022, 12(3), 635; https://doi.org/10.3390/diagnostics12030635 - 4 Mar 2022

Cited by 6 | Viewed by 3387

Abstract

Posterior fossa tumours (PFTs) in infants are very rare, and information on these tumours is scarce in the literature. This retrospective study reports their pathological characteristics and describes surgical aspects and treatment outcomes. A two-centre cohort of infants with PFTs treated from 2007 to 2018 was retrospectively reviewed. Patient characteristics, clinical, and treatment data were reviewed. Survival curves for progression-free survival (PFS) and overall survival (OS) were generated. Thirty-three infants were retrieved. There were 11 low grade and 22 high-grade tumours. The most common presenting symptom was intracranial hypertension. Fifteen children out of thirty-three progressed. Five-year PFS was significantly lower in children with high-grade tumours (38.3%) than those with low-grade tumours (69.3%), p = 0.030. High-grade pathology was the only predictor of progression (HR 3.7, 95% CI 1.1–13.31), p = 0.045. Fourteen children with high-grade tumours died, with a 5-year OS of 55.25%. PFTs in children below one year of age still represent a unique challenge. Infants with high-grade tumours display the worst outcomes and the lowest survival, indicating that more effective strategies are needed. Full article

(This article belongs to the Special Issue Advances in Pediatric Neuro-Oncology 2.0)

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19 pages, 6130 KiB

Open AccessFeature PaperArticle

Atypical Teratoid Rhabdoid Tumours Are Susceptible to Panobinostat-Mediated Differentiation Therapy

by Wai C. Chong, W. Samantha N. Jayasekara, Vijesh G. Vaghjiani, Sarah Parackal, Claire Sun, Dean Popovski, Elizabeth M. Algar, Ron Firestein, Paul J. Wood, Sara Khan, Annie Huang, David M. Ashley, Peter Downie and Jason E. Cain

Cancers 2021, 13(20), 5145; https://doi.org/10.3390/cancers13205145 - 14 Oct 2021

Cited by 6 | Viewed by 3240

Abstract

Atypical teratoid rhabdoid tumour (ATRT) is a rare but highly aggressive undifferentiated solid tumour arising in the central nervous system and predominantly affecting infants and young children. ATRT is exclusively characterized by the inactivation of SMARCB1, a member of the SWI/SNF chromatin remodelling complex that is essential for the regulation of large sets of genes required for normal development and differentiation. Histone deacetylase inhibitors (HDACi) are a promising anticancer therapy and are able to mimic the normal acetylation functions of SMARCB1 in SMARCB1-deficient cells and drive multilineage differentiation in extracranial rhabdoid tumours. However, the potential efficacy of HDACi in ATRT is unknown. Here, we show that human ATRT cells are highly responsive to the HDACi panobinostat and that sustained treatment leads to growth arrest, increased cell senescence, decreased clonogenicity and induction of a neurogenesis gene-expression profile. Furthermore, in an orthotopic ATRT xenograft model, continuous panobinostat treatment inhibits tumour growth, increases survival and drives neuronal differentiation as shown by the expression of the neuronal marker, TUJ1. Collectively, this preclinical study supports the therapeutic potential of panobinostat-mediated differentiation therapy for ATRT. Full article

(This article belongs to the Section Pediatric Oncology)

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18 pages, 4806 KiB

Open AccessReview

Recent Advances in Understanding the Role of Autophagy in Paediatric Brain Tumours

by Francesca Gatto, Giacomo Milletti, Andrea Carai, Angela Mastronuzzi and Francesca Nazio

Diagnostics 2021, 11(3), 481; https://doi.org/10.3390/diagnostics11030481 - 9 Mar 2021

Cited by 8 | Viewed by 4458

Abstract

Autophagy is a degradative process occurring in eukaryotic cells to maintain homeostasis and cell survival. After stressful conditions including nutrient deprivation, hypoxia or drugs administration, autophagy is induced to counteract pathways that could lead to cell death. In cancer, autophagy plays a paradoxical role, acting both as tumour suppressor—by cleaning cells from damaged organelles and inhibiting inflammation or, alternatively, by promoting genomic stability and tumour adaptive response—or as a pro-survival mechanism to protect cells from stresses such as chemotherapy. Neural-derived paediatric solid tumours represent a variety of childhood cancers with unique anatomical location, cellular origins, and clinical presentation. These tumours are a leading cause of morbidity and mortality among children and new molecular diagnostics and therapies are necessary for longer survival and reduced morbidity. Here, we review advances in our understanding of how autophagy modulation exhibits antitumor properties in experimental models of paediatric brain tumours, i.e., medulloblastoma (MB), ependymoma (EPN), paediatric low-grade and high-grade gliomas (LGGs, HGGs), atypical teratoid/rhabdoid tumours (ATRTs), and retinoblastoma (RB). We also discuss clinical perspectives to consider how targeting autophagy may be relevant in these specific paediatric tumours. Full article

(This article belongs to the Special Issue Advances in Pediatric Neuro-Oncology)

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