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Advances in Rhabdomyosarcoma
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Advances in Rhabdomyosarcoma

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: closed (30 November 2021) | Viewed by 8582

Special Issue Editor

Dr. Francesca Megiorni

E-Mail Website
Guest Editor
Department of Experimental Medicine, SAPIENZA University of Rome, 00161 Rome, Italy
Interests: epigenetics; targeted therapy; regenerative medicine; molecular and cellular biology; network medicine; genetics
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Rhabdomyosarcoma (RMS) is the most commonly diagnosed malignant soft tissue tumour in paediatric age, with alveolar RMS (ARMS) and embryonal RMS (ERMS) representing the two main histological subtypes. Despite the improvement in standardized therapeutic protocols, involving surgery, chemotherapy and radiotherapy, patients with metastatic or recurrent disease have a poor clinical outcome with a 5-year overall survival of about 30%. So, the discovery of innovative therapies against RMS is an absolute clinical priority. To this concern, the definition of the specific altered coding and non-coding genes, as well as the precise molecular mechanisms playing role in their aberrant expression and in tumour–microenvironment communication may represent a more focused solution that can help finding new biomarkers and setting novel targeted therapies against RMS. The aim of the present Special Issue is to assemble recent advancements into the in vitro and in vivo effects of a novel generation of antitumoral molecules (epigenetic drugs, microRNAs, small inhibitors), as single agents and in combination with conventional treatments or immunotherapy. Because complex systems, such as malignancies, can be more thoroughly defined if considered as a whole, "omics" approaches will be particularly appreciated for the identification, the characterization and quantification of the drug-related molecular properties. Particular interest will be given to the antitumoral effects against cancer stem cells (CSCs), which have a pivotal role in the acquired therapy resistance associated with RMS progression and relapse, as well as poor clinical outcomes in cancer patients. Rapidly emerging data have the potential to be translated into more efficient and less toxic personalized treatments for the clinical care of RMS patients.

We look forward to your contributions to this Special Issue (Cells; IF: 4.366, ISSN 2073-4409) in the form of original research articles and reviews.

Dr. Francesca Megiorni
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. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • rhabdomyosarcoma
  • targeted therapy
  • antitumoral molecules
  • epigenetics
  • microRNA
  • “omics” analysis
  • biomarkers
  • chemotherapy
  • radiotherapy
  • immunotherapy

Published Papers (3 papers)

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Research

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20 pages, 5947 KiB  
Article
DNMT3A and DNMT3B Targeting as an Effective Radiosensitizing Strategy in Embryonal Rhabdomyosarcoma
by Simona Camero, Giulia Vitali, Paola Pontecorvi, Simona Ceccarelli, Eleni Anastasiadou, Francesca Cicchetti, Elisabetta Flex, Silvia Pomella, Matteo Cassandri, Rossella Rota, Francesco Marampon, Cinzia Marchese, Amalia Schiavetti and Francesca Megiorni
Cells 2021, 10(11), 2956; https://0-doi-org.brum.beds.ac.uk/10.3390/cells10112956 - 30 Oct 2021
Cited by 18 | Viewed by 2365
Abstract
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood. Recently, we demonstrated the overexpression of both DNA methyltransferase 3A (DNMT3A) and 3B (DNMT3B) in RMS tumour biopsies and cell lines compared to normal skeletal muscle. Radiotherapy may often fail due to [...] Read more.
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood. Recently, we demonstrated the overexpression of both DNA methyltransferase 3A (DNMT3A) and 3B (DNMT3B) in RMS tumour biopsies and cell lines compared to normal skeletal muscle. Radiotherapy may often fail due to the abnormal expression of some molecules able to drive resistance mechanisms. The aim of this study was to analyse the involvement of DNMT3A and DNMT3B in radioresistance in RMS. RNA interference experiments against DNMT3A/3B were performed in embryonal RMS cells, upon ionizing radiation (IR) exposure and the effects of the combined treatment on RMS cells were analysed. DNMT3A and DNMT3B knocking down increased the sensitivity of RMS cells to IR, as indicated by the drastic decrease of colony formation ability. Interestingly, DNMT3A/3B act in two different ways: DNMT3A silencing triggers the cellular senescence program by up-regulating p16 and p21, whilst DNMT3B depletion induces significant DNA damage and impairs the DNA repair machinery (ATM, DNA-PKcs and Rad51 reduction). Our findings demonstrate for the first time that DNMT3A and DNMT3B overexpression may contribute to radiotherapy failure, and their inhibition might be a promising radiosensitizing strategy, mainly in the treatment of patients with metastatic or recurrent RMS tumours. Full article
(This article belongs to the Special Issue Advances in Rhabdomyosarcoma)
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25 pages, 8446 KiB  
Article
Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones
by Klaudia Skrzypek, Grażyna Adamek, Marta Kot, Bogna Badyra and Marcin Majka
Cells 2021, 10(8), 1870; https://0-doi-org.brum.beds.ac.uk/10.3390/cells10081870 - 23 Jul 2021
Cited by 3 | Viewed by 2817
Abstract
Rhabdomyosarcoma (RMS), is the most frequent soft tissue tumor in children that originates from disturbances in differentiation process. Mechanisms leading to the development of RMS are still poorly understood. Therefore, by analysis of two RMS RH30 cell line subclones, one subclone PAX7 negative, [...] Read more.
Rhabdomyosarcoma (RMS), is the most frequent soft tissue tumor in children that originates from disturbances in differentiation process. Mechanisms leading to the development of RMS are still poorly understood. Therefore, by analysis of two RMS RH30 cell line subclones, one subclone PAX7 negative, while the second one PAX7 positive, and comparison with other RMS cell lines we aimed at identifying new mechanisms crucial for RMS progression. RH30 subclones were characterized by the same STR profile, but different morphology, rate of proliferation, migration activity and chemotactic abilities in vitro, as well as differences in tumor morphology and growth in vivo. Our analysis indicated a different level of expression of adhesion molecules (e.g., from VLA and ICAM families), myogenic microRNAs, such as miR-206 and transcription factors, such as MYOD, MYOG, SIX1, and ID. Silencing of PAX7 transcription factor with siRNA confirmed the crucial role of PAX7 transcription factor in proliferation, differentiation and migration of RMS cells. To conclude, our results suggest that tumor cell lines with the same STR profile can produce subclones that differ in many features and indicate crucial roles of PAX7 and ID proteins in the development of RMS. Full article
(This article belongs to the Special Issue Advances in Rhabdomyosarcoma)
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Review

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15 pages, 2894 KiB  
Review
HER Tyrosine Kinase Family and Rhabdomyosarcoma: Role in Onset and Targeted Therapy
by Carla De Giovanni, Lorena Landuzzi, Arianna Palladini, Giordano Nicoletti, Patrizia Nanni and Pier-Luigi Lollini
Cells 2021, 10(7), 1808; https://0-doi-org.brum.beds.ac.uk/10.3390/cells10071808 - 16 Jul 2021
Cited by 3 | Viewed by 2667
Abstract
Rhabdomyosarcomas (RMS) are tumors of the skeletal muscle lineage. Two main features allow for distinction between subtypes: morphology and presence/absence of a translocation between the PAX3 (or PAX7) and FOXO1 genes. The two main subtypes are fusion-positive alveolar RMS (ARMS) and fusion-negative embryonal [...] Read more.
Rhabdomyosarcomas (RMS) are tumors of the skeletal muscle lineage. Two main features allow for distinction between subtypes: morphology and presence/absence of a translocation between the PAX3 (or PAX7) and FOXO1 genes. The two main subtypes are fusion-positive alveolar RMS (ARMS) and fusion-negative embryonal RMS (ERMS). This review will focus on the role of receptor tyrosine kinases of the human epidermal growth factor receptor (EGFR) family that is comprised EGFR itself, HER2, HER3 and HER4 in RMS onset and the potential therapeutic targeting of receptor tyrosine kinases. EGFR is highly expressed by ERMS tumors and cell lines, in some cases contributing to tumor growth. If not mutated, HER2 is not directly involved in control of RMS cell growth but can be expressed at significant levels. A minority of ERMS carries a HER2 mutation with driving activity on tumor growth. HER3 is frequently overexpressed by RMS and can play a role in the residual myogenic differentiation ability and in resistance to signaling-directed therapy. HER family members could be exploited for therapeutic approaches in two ways: blocking the HER member (playing a driving role for tumor growth with antibodies or inhibitors) and targeting expressed HER members to vehiculate toxins or immune effectors. Full article
(This article belongs to the Special Issue Advances in Rhabdomyosarcoma)
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