Current Concept and Management of Pediatric ATRTs—2nd Edition

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

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