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http://dx.doi.org/10.1111/nan.12971 | DOI Listing |
Ann Med Surg (Lond)
July 2025
Department of Neurosurgery, Al-istishari Arab Hospital, Ramallah, West Bank, Palestine.
Introduction And Importance: Atypical teratoid rhabdoid tumor (ATRT) is a rare and aggressive central nervous system malignancy. It primarily affects infants and young children, with adult cases being exceptionally uncommon. This case highlights the diagnostic and management challenges posed by ATRT in a 38-year-old pregnant woman.
View Article and Find Full Text PDFFam Cancer
August 2025
Institute of Medical Genetics, School of Medicine, Cardiff University, Cardiff, CF14 4XN, UK.
SMARCB1 is a core unit of the BAF chromatin remodelling complex and its functional impairment interferes with the self-renewal and pluripotency of stem cells, lineage commitment, cellular identity and differentiation. SMARCB1 is also an important tumour suppressor gene and somatic SMARCB1 pathogenic variants (PVs) have been detected in ~ 5% of all human cancers. Additionally, germline SMARCB1 PVs have been identified in patients with conditions as clinically diverse as Rhabdoid Tumour Predisposition Syndrome type 1 (RTPS1), schwannomatosis and neurodevelopmental disorders such as Coffin-Siris syndrome (CSS).
View Article and Find Full Text PDFCancer Med
August 2025
Division of Paediatric Oncology, Tata Memorial Centre, Mumbai, India.
Background: Management of infants and young children with embryonal tumors of the central nervous system remains a challenge. We report the outcomes in these children with the strategy of surgery, pre-irradiation chemotherapy, and delayed radiation.
Procedure: Children less than 3 years and diagnosed with medulloblastoma(MB), atypical teratoid rhabdoid tumor(ATRT), embryonal tumor with multilayered rosettes(ETMR), embryonal tumor tumors NOS(ET NOS) and pineoblastoma(PB), underwent standard evaluation and staging.
ACS Cent Sci
July 2025
Department of Chemistry, Duke University, Durham, North Carolina 27708, United States.
The guaianolide family of sesquiterpene lactones is known for its distinctive structural features and diverse biological activities. 4,9,10-Trihydroxyguaia-11(13)-en-12,6-olide, with an underdetermined absolute stereochemistry ( or -), is a newly identified 6,12-guaianolide isolated from the genus . Motivated by the potential biological activity of the natural product, we pursue its stereoselective synthesis.
View Article and Find Full Text PDFNeuro Oncol
July 2025
Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.
Background: Atypical teratoid rhabdoid tumors (ATRTs) are highly aggressive pediatric central nervous system tumors defined by the inactivation of the SMARCB1 gene. Despite the identification of three distinct molecular subtypes, each defined by unique clinical and molecular characteristics, no subtype-specific therapeutic strategies are currently available. This highlights an urgent need to deepen our understanding of the cellular heterogeneity and developmental origins of ATRTs.
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