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Current Advances in Pediatric Rhabdomyosarcoma
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Current Advances in Pediatric Rhabdomyosarcoma

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Oncology".

Deadline for manuscript submissions: closed (28 February 2021) | Viewed by 17309

Special Issue Editors

Dr. Sanjeev A. Vasudevan

E-Mail Website
Guest Editor
Division of Pediatric Surgery, Texas Children’s Surgical Oncology Program, Baylor College of Medicine, Houston, TX, USA
Interests: pediatric surgical oncology; rhabdomyosarcoma; non-rhabdomyosarcoma soft tissue sarcoma; liver cancer; neuroblastoma; wilms tumor
Dr. Wendy Allen-Rhoades

E-Mail Website
Co-Guest Editor
Division of Pediatric Hematology/Oncology, Mayo Clinic, Rochester, MN 55905, USA
Interests: sarcoma; pediatric oncology; rhabdomyosarcoma; osteosarcoma; ewing sarcoma; non-rhabdomyosarcoma soft tissue sarcoma

Special Issue Information

Dear Colleagues,

The diagnostic approach and treatment of pediatric rhabdomyosarcoma (RMS) has greatly evolved in the last decade through the use of advanced approaches for diagnostics, including refined and new radiologic imaging modalities for staging, and the molecular classification of tumors. Similarly, advances in the treatment of pediatric RMS continue to improve and evolve with refined surgical techniques for local control, advances in radiation oncology modalities, and exploring the role of targeted agents in RMS. 

Historically, alveolar RMS have had inferior outcomes compared to embryonal RMS, and the identification of the PAX/FOXO1 fusion gene has confirmed that the tumors that harbor the PAX/FOXO1 fusion gene continue to have the poorest outcomes. Unfortunately, current treatment regimens do not directly target PAX/FOXO1, but research over the past several years is leading to progress in targeted therapies that may act on other regulators of tumorigenesis in RMS. Further, the conclusion of recent studies has led to changes in practice patterns for risk assignment, dose and duration of chemotherapy regimens, and optimizing local control methods, including the addition of minimally invasive and interventional radiology approaches to RMS. Lastly, we must continue to study environmental and genetic risk factors to identify those most predisposed to developing RMS and support RMS survivors by studying and mitigating late effects of therapy such as neurocognitive outcomes.

This Special Issue of the Journal of Clinical Medicine will focus on the current advances in the diagnosis and management of childhood RMS. We would greatly appreciate your contribution to this Special Edition by offering a review article or original scientific work related to this topic.

Sincerely,

Dr. Sanjeev Vasudevan
Dr. Wendy Allen-Rhoades
Guest Editors

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. Journal of Clinical Medicine 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 2600 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
  • soft tissue sarcoma
  • local control for rhabdomyosarcoma
  • systemic therapy for rhabdomyosarcoma

Published Papers (4 papers)

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Research

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14 pages, 3656 KiB  
Article
Endoglin Expression and Microvessel Density as Prognostic Factors in Pediatric Rhabdomyosarcoma
by Joanna Radzikowska, Antoni Krzeski, Anna M. Czarnecka, Teresa Klepacka, Magdalena Rychlowska-Pruszynska, Anna Raciborska, Bozenna Dembowska-Baginska, Maciej Pronicki, Andrzej Kukwa, Janusz Sierdzinski and Wojciech Kukwa
J. Clin. Med. 2021, 10(3), 512; https://doi.org/10.3390/jcm10030512 - 01 Feb 2021
Cited by 7 | Viewed by 2025
Abstract
(1) Background: The study proposed to analyze microvessel density (MVD) in rhabdomyosarcoma (RMS) based on the expression of angiogenesis markers and define its prognostic role in this group of patients. (2) Methods: The study included forty-nine pediatric patients diagnosed with RMS. Tumor tissue [...] Read more.
(1) Background: The study proposed to analyze microvessel density (MVD) in rhabdomyosarcoma (RMS) based on the expression of angiogenesis markers and define its prognostic role in this group of patients. (2) Methods: The study included forty-nine pediatric patients diagnosed with RMS. Tumor tissue expression of CD31, CD34, and CD105 was analyzed. MVD was calculated and correlated with clinical RMS prognostic parameters. (3) Results: CD31, CD34, and CD105 are expressed in all RMS cases. MVD/CD105 was significantly higher in the RMS group than in the control group. The mean and median values of MVD/CD105 in RMS were lower than MVD/CD31 and MVD/CD34. MVD/CD105 was significantly higher in patients with alveolar RMS and those with metastatic disease. Patients with higher levels of MVD/CD105 had a higher risk of death (HR = 1.009). (4) Conclusion: CD105 is a relevant angiogenesis marker in pediatric RMS, and MVD/CD105 is an independent risk factor of short overall survival in children with RMS. Full article
(This article belongs to the Special Issue Current Advances in Pediatric Rhabdomyosarcoma)
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Review

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17 pages, 1030 KiB  
Review
Pediatric Rhabdomyosarcoma: Epidemiology and Genetic Susceptibility
by Bailey A. Martin-Giacalone, P. Adam Weinstein, Sharon E. Plon and Philip J. Lupo
J. Clin. Med. 2021, 10(9), 2028; https://doi.org/10.3390/jcm10092028 - 09 May 2021
Cited by 24 | Viewed by 6210
Abstract
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children, yet little is known about its etiology. Studies that examine either environmental exposures or germline genetic predisposition in RMS have begun to identify factors that contribute to this malignancy. Here, we summarize epidemiological [...] Read more.
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children, yet little is known about its etiology. Studies that examine either environmental exposures or germline genetic predisposition in RMS have begun to identify factors that contribute to this malignancy. Here, we summarize epidemiological reports of RMS incidence in terms of several factors, including age at diagnosis, biological sex, and geographic location. We then describe findings from association studies, which explore the role of parental exposures, birth and perinatal characteristics, and childhood exposures in RMS. Further, we discuss RMS predisposition syndromes and large-scale sequencing studies that have further identified RMS-associated genes. Finally, we propose future directions of study, which aim to advance our understanding of the origin of RMS and can provide knowledge for novel RMS therapies. Full article
(This article belongs to the Special Issue Current Advances in Pediatric Rhabdomyosarcoma)
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17 pages, 493 KiB  
Review
Prioritization of Novel Agents for Patients with Rhabdomyosarcoma: A Report from the Children’s Oncology Group (COG) New Agents for Rhabdomyosarcoma Task Force
by Holly L. Pacenta, Wendy Allen-Rhoades, David Langenau, Peter J. Houghton, Charles Keller, Christine M. Heske, Michael D. Deel, Corinne M. Linardic, Jack F. Shern, Elizabeth Stewart, Brian Turpin, Douglas J. Harrison, Javed Khan, Leo Mascarenhas, Stephen X. Skapek, William H. Meyer, Douglas S. Hawkins, Eleanor Y. Chen, James F. Amatruda, Pooja Hingorani and Theodore W. Laetschadd Show full author list remove Hide full author list
J. Clin. Med. 2021, 10(7), 1416; https://doi.org/10.3390/jcm10071416 - 01 Apr 2021
Cited by 13 | Viewed by 4252
Abstract
Rhabdomyosarcoma is the most common soft tissue sarcoma diagnosed in children and adolescents. Patients that are diagnosed with advanced or relapsed disease have exceptionally poor outcomes. The Children’s Oncology Group (COG) convened a rhabdomyosarcoma new agent task force in 2020 to systematically evaluate [...] Read more.
Rhabdomyosarcoma is the most common soft tissue sarcoma diagnosed in children and adolescents. Patients that are diagnosed with advanced or relapsed disease have exceptionally poor outcomes. The Children’s Oncology Group (COG) convened a rhabdomyosarcoma new agent task force in 2020 to systematically evaluate novel agents for inclusion in phase 2 or phase 3 clinical trials for patients diagnosed with rhabdomyosarcoma, following a similar effort for Ewing sarcoma. The task force was comprised of clinicians and basic scientists who collectively identified new agents for evaluation and prioritization in clinical trial testing. Here, we report the work of the task force including the framework upon which the decisions were rendered and review the top classes of agents that were discussed. Representative agents include poly-ADP-ribose polymerase (PARP) inhibitors in combination with cytotoxic agents, mitogen-activated protein kinase (MEK) inhibitors in combination with type 1 insulin-like growth factor receptor (IGFR1) inhibitors, histone deacetylase (HDAC) inhibitors, and novel cytotoxic agents. Full article
(This article belongs to the Special Issue Current Advances in Pediatric Rhabdomyosarcoma)
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15 pages, 294 KiB  
Review
Relapsed Rhabdomyosarcoma
by Christine M. Heske and Leo Mascarenhas
J. Clin. Med. 2021, 10(4), 804; https://doi.org/10.3390/jcm10040804 - 17 Feb 2021
Cited by 31 | Viewed by 4113
Abstract
Relapsed rhabdomyosarcoma (RMS) represents a significant therapeutic challenge. Nearly one-third of patients diagnosed with localized RMS and over two-thirds of patients with metastatic RMS will experience disease recurrence following primary treatment, generally within three years. Clinical features at diagnosis, including primary site, tumor [...] Read more.
Relapsed rhabdomyosarcoma (RMS) represents a significant therapeutic challenge. Nearly one-third of patients diagnosed with localized RMS and over two-thirds of patients with metastatic RMS will experience disease recurrence following primary treatment, generally within three years. Clinical features at diagnosis, including primary site, tumor invasiveness, size, stage, and histology impact likelihood of relapse and prognosis post-relapse. Aspects of initial treatment, including extent of surgical resection, use of radiotherapy, and chemotherapy regimen, are also associated with post-relapse outcomes, as are features of the relapse itself, including time to relapse and extent of disease involvement. Although there is no standard treatment for patients with relapsed RMS, several general principles, including tissue biopsy confirmation of diagnosis, assessment of post-relapse prognosis, determination of the feasibility of additional local control measures, and discussion of patient goals, should all be part of the approach to care. Patients with features suggestive of a favorable prognosis, which include those with botryoid RMS or stage 1 or group I embryonal RMS (ERMS) who have had no prior treatment with cyclophosphamide, have the highest chance of achieving long-term cure when treated with a multiagent chemotherapy regimen at relapse. Unfortunately, patients who do not meet these criteria represent the majority and have poor outcomes when treated with such regimens. For this group, strong consideration should be given for enrollment on a clinical trial. Full article
(This article belongs to the Special Issue Current Advances in Pediatric Rhabdomyosarcoma)
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