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Current Concept and Management of Pediatric ATRTs—2nd Edition
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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: closed (30 April 2026) | Viewed by 3161

Special Issue Editors

Prof. Dr. Tadanori Tomita

E-Mail Website
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
Dr. Alicia C. Lenzen

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 (3 papers)

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25 pages, 10991 KB  
Article
Histogenesis of Atypical Teratoid Rhabdoid Tumors: Anatomical and Embryological Perspectives
by Tadanori Tomita
Cancers 2026, 18(1), 8; https://doi.org/10.3390/cancers18010008 - 19 Dec 2025
Viewed by 865
Abstract
Objectives: Atypical teratoid/rhabdoid tumors (ATRTs) are rare, malignant central nervous system (CNS) neoplasms that predominantly affect infants and young children. While ATRT arises throughout the CNS, its extracranial counterpart, malignant rhabdoid tumor, occurs in other organs. A single-institutional cohort is reviewed to map [...] Read more.
Objectives: Atypical teratoid/rhabdoid tumors (ATRTs) are rare, malignant central nervous system (CNS) neoplasms that predominantly affect infants and young children. While ATRT arises throughout the CNS, its extracranial counterpart, malignant rhabdoid tumor, occurs in other organs. A single-institutional cohort is reviewed to map anatomic distribution of pediatric ATRTs and to integrate a literature review to contextualize ATRT histogenesis from anatomical and embryological perspectives. Methods: A retrospective review was conducted on a cohort of 50 pediatric patients with ATRT treated over 20 years. Demographic, surgical, and neuroimaging data were correlated to define tumor location, extent, and compartmental involvement. A focused literature review synthesized molecular subclassifications and proposed cells of origin/cytogenesis. Results: Of the 50 ATRTs, 18 (36%) were infratentorial, 15 (30%) supratentorial, 11 (22%) in the pineal region, and 6 (12%) in the spinal compartment. Among infratentorial tumors, 10 were centered in the fourth ventricle, with or without extension into the cerebellopontine angle (CPA) cistern; the remainder arose in the CPA. Among ATRTs of the cerebral hemispheres, 3 showed bi-hemispheric involvement crossing the falx cerebri. ATRTs of the pineal region predominantly originated from the superior medullary velum. These topographic data were corelated with embryological and molecular information available in the literature. Conclusions: ATRTs arise across diverse neuroanatomical compartments—including intraparenchymal, intraventricular, extra-axial, and extradural sites—underscoring biological heterogeneity. Inactivation of SMARCB1 is the defining molecular event and principal oncogenic driver, although the upstream mechanisms precipitating these alterations remain incompletely resolved. Molecular subgroups—ATRT-TYR, ATRT-SHH, and ATRT-MYC—display distinct age distributions and anatomic predilections, implicating developmental context in tumor initiation. The characteristic cellular admixture of rhabdoid cells with mesenchymal and/or epithelial differentiation, together with intra- and extra-axial and occasional extradural presentations, supports a model in which at least a subset of ATRTs may originate from neural crest-derived lineages, despite little or no neural crest contribution to brain parenchyma development. Neural plate border progenitors with bipotent features represent a plausible intraparenchymal cell of origin. Definitive resolution of these origins and the mechanisms of SMARCB1 disruption will require integrated approaches. Further investigations are warranted to clarify these mechanisms. Full article
(This article belongs to the Special Issue Current Concept and Management of Pediatric ATRTs—2nd Edition)
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Review

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19 pages, 552 KB  
Review
Pediatric Spinal Atypical Teratoid Rhabdoid Tumor: Recent Advances in Biology and Management Options
by Ruby Siada, Kaushik Banerjee, Payal Malhotra, Mohannad Ibrahim, Daniel C. Moreira, John R. Prensner and Santhosh A. Upadhyaya
Cancers 2026, 18(7), 1171; https://doi.org/10.3390/cancers18071171 - 5 Apr 2026
Viewed by 742
Abstract
Atypical teratoid rhabdoid tumor (AT/RT) is a rare, highly aggressive embryonal central nervous system malignancy occurring predominately in infants and toddlers. Spinal AT/RT (spAT/RT) cases are even more limited, and as a result, little is known regarding prognostic factors and optimal treatment regimens. [...] Read more.
Atypical teratoid rhabdoid tumor (AT/RT) is a rare, highly aggressive embryonal central nervous system malignancy occurring predominately in infants and toddlers. Spinal AT/RT (spAT/RT) cases are even more limited, and as a result, little is known regarding prognostic factors and optimal treatment regimens. Molecularly, AT/RT is divided into three groups: AT/RT-SHH, AT/RT-TYR and AT/RT-MYC. spAT/RT is predominantly of the MYC subtype. Additionally, a third of patients with AT/RT have a germline Rhabdoid Tumor Predisposition Syndrome (RTPS) that increases the likelihood of developing additional rhabdoid tumors, including renal rhabdoid tumors. Due to the rarity of these tumors, there is a lack of consensus on treatment strategies to be employed. This review paper details the published literature on spAT/RT, with particular emphasis on the recent advances in understanding the biology of these aggressive tumors and currently available therapeutic options, and highlights the challenges associated with the management of this extremely rare condition. Full article
(This article belongs to the Special Issue Current Concept and Management of Pediatric ATRTs—2nd Edition)
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Other

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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
Cited by 1 | Viewed by 1129
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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