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Molecular Determinants of Neuroblastoma
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Molecular Determinants of Neuroblastoma

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Oncology".

Deadline for manuscript submissions: closed (15 July 2021) | Viewed by 18149

Special Issue Editor

Dr. Fabio Morandi

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Guest Editor
IRCCS Istituto Giannina Gaslini - Ospedale Pediatrico, Genoa, Italy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Neuroblastoma (NB) is the most common pediatric extracranial solid tumor, which usually occurs in children under 5 years of age. On the basis of clinical parameters, NB patients are classified as i) low-risk patients, which display localized tumors, with a good response to standard treatment and frequent spontaneous regression, or ii) high-risk patients, which usally disaplay metastatic tumors, with a high frequency of relapse and a survival less than 50%. This Special Issue is focused on molecular determinants that are involved in i) malignant transformation of neuroblasts, ii) metastatic spread and iii) response to chemotherapy in NB patients

Dr. Fabio Morandi
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

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Related Special Issue

Published Papers (7 papers)

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Editorial

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4 pages, 464 KiB  
Editorial
Molecular Determinants of Neuroblastoma
by Fabio Morandi
Int. J. Mol. Sci. 2022, 23(7), 3751; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms23073751 - 29 Mar 2022
Cited by 1 | Viewed by 1500
Abstract
The aim of this Special Issue was to discuss novel findings regarding the different mechanisms involved in the progression of neuroblastoma (NB), which represents the most common pediatric extra-cranial solid tumor [...] Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
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Research

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10 pages, 2001 KiB  
Article
The Anti-Tumor Activity of the NEDD8 Inhibitor Pevonedistat in Neuroblastoma
by Jennifer H. Foster, Eveline Barbieri, Linna Zhang, Kathleen A. Scorsone, Myrthala Moreno-Smith, Peter Zage and Terzah M. Horton
Int. J. Mol. Sci. 2021, 22(12), 6565; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22126565 - 18 Jun 2021
Cited by 6 | Viewed by 2498
Abstract
Pevonedistat is a neddylation inhibitor that blocks proteasomal degradation of cullin–RING ligase (CRL) proteins involved in the degradation of short-lived regulatory proteins, including those involved with cell-cycle regulation. We determined the sensitivity and mechanism of action of pevonedistat cytotoxicity in neuroblastoma. Pevonedistat cytotoxicity [...] Read more.
Pevonedistat is a neddylation inhibitor that blocks proteasomal degradation of cullin–RING ligase (CRL) proteins involved in the degradation of short-lived regulatory proteins, including those involved with cell-cycle regulation. We determined the sensitivity and mechanism of action of pevonedistat cytotoxicity in neuroblastoma. Pevonedistat cytotoxicity was assessed using cell viability assays and apoptosis. We examined mechanisms of action using flow cytometry, bromodeoxyuridine (BrDU) and immunoblots. Orthotopic mouse xenografts of human neuroblastoma were generated to assess in vivo anti-tumor activity. Neuroblastoma cell lines were very sensitive to pevonedistat (IC50 136–400 nM). The mechanism of pevonedistat cytotoxicity depended on p53 status. Neuroblastoma cells with mutant (p53MUT) or reduced levels of wild-type p53 (p53si-p53) underwent G2-M cell-cycle arrest with rereplication, whereas p53 wild-type (p53WT) cell lines underwent G0-G1 cell-cycle arrest and apoptosis. In orthotopic neuroblastoma models, pevonedistat decreased tumor weight independent of p53 status. Control mice had an average tumor weight of 1.6 mg + 0.8 mg versus 0.5 mg + 0.4 mg (p < 0.05) in mice treated with pevonedistat. The mechanism of action of pevonedistat in neuroblastoma cell lines in vitro appears p53 dependent. However, in vivo studies using mouse neuroblastoma orthotopic models showed a significant decrease in tumor weight following pevonedistat treatment independent of the p53 status. Novel chemotherapy agents, such as the NEDD8-activating enzyme (NAE) inhibitor pevonedistat, deserve further study in the treatment of neuroblastoma. Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
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14 pages, 4556 KiB  
Article
MIAT Is an Upstream Regulator of NMYC and the Disruption of the MIAT/NMYC Axis Induces Cell Death in NMYC Amplified Neuroblastoma Cell Lines
by Barbara Feriancikova, Tereza Feglarova, Lenka Krskova, Tomas Eckschlager, Ales Vicha and Jan Hrabeta
Int. J. Mol. Sci. 2021, 22(7), 3393; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22073393 - 25 Mar 2021
Cited by 6 | Viewed by 1774
Abstract
Neuroblastoma (NBL) is the most common extracranial childhood malignant tumor and represents a major cause of cancer-related deaths in infants. NMYC amplification or overexpression is associated with the malignant behavior of NBL tumors. In the present study, we revealed an association between long [...] Read more.
Neuroblastoma (NBL) is the most common extracranial childhood malignant tumor and represents a major cause of cancer-related deaths in infants. NMYC amplification or overexpression is associated with the malignant behavior of NBL tumors. In the present study, we revealed an association between long non-coding RNA (lncRNA) myocardial infarction associated transcript (MIAT) and NMYC amplification in NBL cell lines and MIAT expression in NBL tissue samples. MIAT silencing induces cell death only in cells with NMYC amplification, but in NBL cells without NMYC amplification it decreases only the proliferation. MIAT downregulation markedly reduces the NMYC expression in NMYC-amplified NBL cell lines and c-Myc expression in NMYC non-amplified NBL cell lines, but the ectopic overexpression or downregulation of NMYC did not affect the expression of MIAT. Moreover, MIAT downregulation results in decreased ornithine decarboxylase 1 (ODC1), a known transcriptional target of MYC oncogenes, and decreases the glycolytic metabolism and respiratory function. These results indicate that MIAT is an upstream regulator of NMYC and that MIAT/NMYC axis disruption induces cell death in NMYC-amplified NBL cell lines. These findings reveal a novel mechanism for the regulation of NMYC in NBL, suggesting that MIAT might be a potential therapeutic target, especially for those with NMYC amplification. Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
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Review

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20 pages, 999 KiB  
Review
Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
by Anna M. Wulf, Marcela M. Moreno, Chloé Paka, Alexandra Rampasekova and Karen J. Liu
Int. J. Mol. Sci. 2021, 22(21), 11718; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms222111718 - 29 Oct 2021
Cited by 12 | Viewed by 4872
Abstract
Neuroblastoma is a common extracranial solid tumour of childhood, responsible for 15% of cancer-related deaths in children. Prognoses vary from spontaneous remission to aggressive disease with extensive metastases, where treatment is challenging. Tumours are thought to arise from sympathoadrenal progenitor cells, which derive [...] Read more.
Neuroblastoma is a common extracranial solid tumour of childhood, responsible for 15% of cancer-related deaths in children. Prognoses vary from spontaneous remission to aggressive disease with extensive metastases, where treatment is challenging. Tumours are thought to arise from sympathoadrenal progenitor cells, which derive from an embryonic cell population called neural crest cells that give rise to diverse cell types, such as facial bone and cartilage, pigmented cells, and neurons. Tumours are found associated with mature derivatives of neural crest, such as the adrenal medulla or paraspinal ganglia. Sympathoadrenal progenitor cells express anaplastic lymphoma kinase (ALK), which encodes a tyrosine kinase receptor that is the most frequently mutated gene in neuroblastoma. Activating mutations in the kinase domain are common in both sporadic and familial cases. The oncogenic role of ALK has been extensively studied, but little is known about its physiological role. Recent studies have implicated ALK in neural crest migration and sympathetic neurogenesis. However, very few downstream targets of ALK have been identified. Here, we describe pathological activation of ALK in the neural crest, which promotes proliferation and migration, while preventing differentiation, thus inducing the onset of neuroblastoma. Understanding the effects of ALK activity on neural crest cells will help find new targets for neuroblastoma treatment. Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
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16 pages, 298 KiB  
Review
Pharmacogenetics in Neuroblastoma: What Can Already Be Clinically Implemented and What Is Coming Next?
by Gladys G. Olivera, Andrea Urtasun, Luis Sendra, Salvador F. Aliño, Yania Yáñez, Vanessa Segura, Pablo Gargallo, Pablo Berlanga, Victoria Castel, Adela Cañete and María José Herrero
Int. J. Mol. Sci. 2021, 22(18), 9815; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22189815 - 10 Sep 2021
Cited by 5 | Viewed by 1968
Abstract
Pharmacogenetics is one of the cornerstones of Personalized Precision Medicine that needs to be implemented in the routine of our patients’ clinical management in order to tailor their therapies as much as possible, with the aim of maximizing efficacy and minimizing toxicity. This [...] Read more.
Pharmacogenetics is one of the cornerstones of Personalized Precision Medicine that needs to be implemented in the routine of our patients’ clinical management in order to tailor their therapies as much as possible, with the aim of maximizing efficacy and minimizing toxicity. This is of great importance, especially in pediatric cancer and even more in complex malignancies such as neuroblastoma, where the rates of therapeutic success are still below those of many other types of tumors. The studies are mainly focused on germline genetic variants and in the present review, state of the art is presented: which are the variants that have a level of evidence high enough to be implemented in the clinic, and how to distinguish them from the ones that still need validation to confirm their utility. Further aspects as relevant characteristics regarding ontogeny and future directions in the research will also be discussed. Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
13 pages, 500 KiB  
Review
An Overview of Long Non-Coding (lnc)RNAs in Neuroblastoma
by Francesca Baldini, Matilde Calderoni, Laura Vergani, Paola Modesto, Tullio Florio and Aldo Pagano
Int. J. Mol. Sci. 2021, 22(8), 4234; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22084234 - 19 Apr 2021
Cited by 11 | Viewed by 2627
Abstract
Neuroblastoma (NB) is a heterogeneous developmental tumor occurring in childhood, which arises from the embryonic sympathoadrenal cells of the neural crest. Although the recent progress that has been done on this tumor, the mechanisms involved in NB are still partially unknown. Despite some [...] Read more.
Neuroblastoma (NB) is a heterogeneous developmental tumor occurring in childhood, which arises from the embryonic sympathoadrenal cells of the neural crest. Although the recent progress that has been done on this tumor, the mechanisms involved in NB are still partially unknown. Despite some genetic aberrations having been identified, the sporadic cases represent the majority. Due to its wide heterogeneity in clinical behavior and etiology, NB represents a challenge in terms of prevention and treatment. Since a definitive therapy is lacking so far, there is an urgent necessity to unveil the molecular mechanisms behind NB onset and progression to develop new therapeutic approaches. Long non-coding RNAs (lncRNAs) are a group of RNAs longer than 200 nucleotides. Whether lncRNAs are destined to become a protein or not, they exert multiple biological functions such as regulating gene expression and functions. In recent decades, different research has highlighted the possible role of lncRNAs in the pathogenesis of many diseases, including cancer. Moreover, lncRNAs may represent potential markers or targets for diagnosis and treatment of diseases. This mini-review aimed to briefly summarize the most recent findings on the involvement of some lncRNAs in NB disease by focusing on their mechanisms of action and possible role in unveiling NB onset and progression. Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
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Graphical abstract

14 pages, 1184 KiB  
Review
The Role of Extracellular Vesicles in the Progression of Human Neuroblastoma
by Danilo Marimpietri, Irma Airoldi, Angelo Corso Faini, Fabio Malavasi and Fabio Morandi
Int. J. Mol. Sci. 2021, 22(8), 3964; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22083964 - 12 Apr 2021
Cited by 10 | Viewed by 1965
Abstract
The long-underestimated role of extracellular vesicles in cancer is now reconsidered worldwide by basic and clinical scientists, who recently highlighted novel and crucial activities of these moieties. Extracellular vesicles are now considered as king transporters of specific cargoes, including molecular components of parent [...] Read more.
The long-underestimated role of extracellular vesicles in cancer is now reconsidered worldwide by basic and clinical scientists, who recently highlighted novel and crucial activities of these moieties. Extracellular vesicles are now considered as king transporters of specific cargoes, including molecular components of parent cells, thus mediating a wide variety of cellular activities both in normal and neoplastic tissues. Here, we discuss the multifunctional activities and underlying mechanisms of extracellular vesicles in neuroblastoma, the most frequent common extra-cranial tumor in childhood. The ability of extracellular vesicles to cross-talk with different cells in the tumor microenvironment and to modulate an anti-tumor immune response, tumorigenesis, tumor growth, metastasis and drug resistance will be pinpointed in detail. The results obtained on the role of extracellular vesicles may represent a panel of suggestions potentially useful in practice, due to their involvement in the response to chemotherapy, and, moreover, their ability to predict resistance to standard therapies—all issues of clinical relevance. Full article
(This article belongs to the Special Issue Molecular Determinants of Neuroblastoma)
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