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Current Concept and Management of Pediatric ATRTs—2nd Edition

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

Deadline for manuscript submissions: 30 April 2026 | Viewed by 330

Special Issue Editors


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

E-Mail Website
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,

This Special Issue is the second edition of a previous one, entitled "Current Concept and Management of Pediatric ATRTs" (https://www.mdpi.com/journal/cancers/special_issues/K71523QG63).

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

Since their first description by Rorke in 1995, the histogenesis of ATRTs 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 also been proposed. The WHO Classification of CNS tumors has included ATRTs in “Embryonal Tumors”. ATRTs are distinct from other embryonal tumors in their clinical presentation, tumor location, imaging characteristics, and prognosis. ATRTs often represent a surgical challenge as treatment requires removing a deep-seated large vascular mass from young patients. They are often resistant to conventional chemotherapy. Radiation therapy, which is imperative, raises serious concerns with regard to effects on the developing CNS of young patients. Safe and effective therapeutic measures are urgently needed.

This Special Issue will highlight recent advances in the biological understanding and clinical management of ATRTs derived from preclinical, translational, and/or clinical investigations. The results of innovative diagnostic modalities (molecular and imaging) will be included. Descriptions of results from 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, as well as 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 250 words) can be sent to the Editorial Office for assessment.

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.

Keywords

  • atypical teratoid rhabdoid tumors (ATRTs)
  • CNS tumor
  • embryonal tumors

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Published Papers (1 paper)

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10 pages, 12569 KB  
Opinion
Atypical Teratoid Rhabdoid Tumor: How Tumor Diagnostic Methods in the Laboratory Have Evolved over the Past 40 Years
by Heather L. Smith, Pascale Aouad and Nitin R. Wadhwani
Cancers 2025, 17(23), 3768; https://doi.org/10.3390/cancers17233768 - 26 Nov 2025
Viewed by 213
Abstract
Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a highly aggressive neoplasm of the central nervous system (CNS), most commonly affecting infants and young children. Originally recognized as a distinct entity following cytogenetic identification of monosomy 22 in renal Rhabdoid Tumors, AT/RT now encompasses CNS tumors [...] Read more.
Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a highly aggressive neoplasm of the central nervous system (CNS), most commonly affecting infants and young children. Originally recognized as a distinct entity following cytogenetic identification of monosomy 22 in renal Rhabdoid Tumors, AT/RT now encompasses CNS tumors characterized by SMARCB1 (INI-1) or SMARCA4 (BRG-1) alterations within the SWI/SNF chromatin-remodeling complex. The integration of immunohistochemical markers with advanced molecular diagnostics—including next-generation sequencing, DNA methylation profiling, and gene enrichment analyses—has facilitated robust tumor classification and the identification of three molecular subgroups: TYR, SHH, and MYC. Despite its distinctive histopathologic features, AT/RT remains diagnostically challenging in adolescent and adult populations due to age-related bias and potential morphologic heterogeneity. Differential considerations, including epithelioid sarcoma, poorly differentiated chordoma, CRINET, choroid plexus carcinoma, and rare composite tumors, further complicate the diagnostic landscape. A comprehensive, multimodal diagnostic approach combining histologic, immunophenotypic, and molecular data is essential to accurately identify AT/RT and guide clinical management, particularly in diagnostically ambiguous or atypical cases. Full article
(This article belongs to the Special Issue Current Concept and Management of Pediatric ATRTs—2nd Edition)
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