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: 30 April 2024 | Viewed by 1023
Special Issue Editors
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
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
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