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Cancers
Special Issues
Current Concept and Management of Pediatric ATRTs
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Current Concept and Management of Pediatric ATRTs

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Pediatric Oncology".

Deadline for manuscript submissions: 31 July 2025 | Viewed by 3052

Special Issue Editors

Prof. Dr. Tadanori Tomita

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Guest Editor
Division of Pediatric Neurosurgery, Ann and Robert H. Lurie Children's Hospital, Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Interests: pediatric neurosurgery; congenital CNS malformations; neuro-oncology; developmental biology; spine and spinal cord tumors; spinal tumors
Special Issues, Collections and Topics in MDPI journals
Dr. Alicia C. Lenzen

E-Mail Website
Co-Guest Editor
Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital, Chicago, IL, USA
Interests: pediatric neuro-oncology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Atypical teratoid rhabdoid tumors (ATRTs) are one of the CNS tumors that affect primarily infants and young children. They are fast-growing, invasive tumors with a high propensity of CSF disseminations. ATRTs occur in every location of the brain and spinal cord and present with a high case mortality following current treatment moderlities.

Since the first description by Rorke in 1995, the histogenesis of ATRT has remained an enigma.  Due to the expression of neural, epithelial, and mesenchymal markers, they likely derive from pluripotent fetal cells.  Based on genetic and DNA methylation status and transcriptome profiles, ATRTs are further divided into three distinct molecular subgroups: ATRT-SHH, ATRT-TYR, and ATRT-MYC; further subclassifications have been proposed. The WHO Classification of the CNS tumors has included ATRTs in “Embryonal Tumors”.  ATRTs are distinct from other embryonal tumors because of their clinical presentation, tumor location, imaging characteristics, and prognosis.  ATRTs are often a surgical challenge when extirpating a deep-seated large vascular mass in young patients.  They are often resistant to conventional  chemotherapy. Radiation therapy, which is imperative, raises serious concerns around affecting the developing CNS of the young. Safe and effective therapeutic measures are desperately needed.

This Special Issue will highlight recent advances in the biological understanding and clinical management of ATRT, deriving from preclinical, translational and/or clinical investigations. The results of innovative diagnostic modalities (molecular and imaging) will be included.  The descriptions of results of case series or multi-center group studies are encouraged for a better understanding of the clinical behavior of these tumors.   Reports of advanced surgical techniques for resection and also adjuvant therapy including novel molecular-targeted therapy and/or immunotherapy are welcome.

Prof. Dr. Tadanori Tomita
Dr. Alicia C. Lenzen
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 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. Cancers 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 2900 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.

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Published Papers (2 papers)

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15 pages, 1670 KiB  
Article
What Have We Learnt from the Recent Multimodal Managements of Young Patients with ATRT?
by Sylvia Cheng, Chantel Cacciotti, Carol L. S. Yan and Lucie Lafay-Cousin
Cancers 2025, 17(7), 1116; https://doi.org/10.3390/cancers17071116 - 26 Mar 2025
Viewed by 360
Abstract
Atypical teratoid rhabdoid tumors are rare embryonal tumors of the nervous system mainly seen in very young children with aggressive behavior and dismal prognosis when treated with conventional chemotherapy only. More recent multimodal strategies combining, variably, high dose chemotherapy, radiotherapy and or intrathecal [...] Read more.
Atypical teratoid rhabdoid tumors are rare embryonal tumors of the nervous system mainly seen in very young children with aggressive behavior and dismal prognosis when treated with conventional chemotherapy only. More recent multimodal strategies combining, variably, high dose chemotherapy, radiotherapy and or intrathecal chemotherapy have led to some stride in survival. We present the results of the most recent clinical trials and registry data for patients treated with these multimodal approaches with survival ranging from 37.1% to 88.9%. We review the current consensus of the molecular characterization of these tumors into 3 subgroups (ATRT-TYR, ATRT-SHH and ATRT-MYC) and discuss the potential clinical impact of molecular subgrouping on survival. We explore other therapeutic tools including intrathecal chemotherapy and maintenance and possible new targeted agents for patients failing multimodal strategies Full article
(This article belongs to the Special Issue Current Concept and Management of Pediatric ATRTs)
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16 pages, 7070 KiB  
Article
Dynamic Survival Risk Prognostic Model and Genomic Landscape for Atypical Teratoid/Rhabdoid Tumors: A Population-Based, Real-World Study
by Sihao Chen, Yi He, Jiao Liu, Ruixin Wu, Menglei Wang and Aishun Jin
Cancers 2024, 16(5), 1059; https://doi.org/10.3390/cancers16051059 - 5 Mar 2024
Viewed by 1938
Abstract
Background: An atypical teratoid/rhabdoid tumor (AT/RT) is an uncommon and aggressive pediatric central nervous system neoplasm. However, a universal clinical consensus or reliable prognostic evaluation system for this malignancy is lacking. Our study aimed to develop a risk model based on comprehensive clinical [...] Read more.
Background: An atypical teratoid/rhabdoid tumor (AT/RT) is an uncommon and aggressive pediatric central nervous system neoplasm. However, a universal clinical consensus or reliable prognostic evaluation system for this malignancy is lacking. Our study aimed to develop a risk model based on comprehensive clinical data to assist in clinical decision-making. Methods: We conducted a retrospective study by examining data from the Surveillance, Epidemiology, and End Results (SEER) repository, spanning 2000 to 2019. The external validation cohort was sourced from the Children’s Hospital Affiliated to Chongqing Medical University, China. To discern independent factors affecting overall survival (OS) and cancer-specific survival (CSS), we applied Least Absolute Shrinkage and Selection Operator (LASSO) and Random Forest (RF) regression analyses. Based on these factors, we structured nomogram survival predictions and initiated a dynamic online risk-evaluation system. To contrast survival outcomes among diverse treatments, we used propensity score matching (PSM) methodology. Molecular data with the most common mutations in AT/RT were extracted from the Catalogue of Somatic Mutations in Cancer (COSMIC) database. Results: The annual incidence of AT/RT showed an increasing trend (APC, 2.86%; 95% CI:0.75–5.01). Our prognostic study included 316 SEER database participants and 27 external validation patients. The entire group had a median OS of 18 months (range 11.5 to 24 months) and median CSS of 21 months (range 11.7 to 29.2). Evaluations involving C-statistics, DCA, and ROC analysis underscored the distinctive capabilities of our prediction model. An analysis via PSM highlighted that individuals undergoing triple therapy (integrating surgery, radiotherapy, and chemotherapy) had discernibly enhanced OS and CSS. The most common mutations of AT/RT identified in the COSMIC database were SMARCB1, BRAF, SMARCA4, NF2, and NRAS. Conclusions: In this study, we devised a predictive model that effectively gauges the prognosis of AT/RT and briefly analyzed its genomic features, which might offer a valuable tool to address existing clinical challenges. Full article
(This article belongs to the Special Issue Current Concept and Management of Pediatric ATRTs)
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