Background: Primary brain tumor is a leading cause of death in cancer-bearing children. Acutely progressive patterns of electroencephalography (EEG) remain to be investigated for children with rapidly growing brain tumors.

Case Report: A 14-month-old boy was transferred to our department for prolonged seizures and unrecovered consciousness on his fifth day of illness. The EEG recording on admission showed highly disorganized background activity with high-voltage rhythmic delta waves. Serial EEG monitoring revealed a rapid transition of the background activity to the suppression-burst pattern, and then to generalized suppression of cortical activity within a few hours after admission. Magnetic resonance imaging detected a midline tumor at the pineal gland extending to the midbrain and pons. The tumor was pathologically confirmed as atypical teratoid/rhabdoid tumor (AT/RT) with absent expression of SMARCB1. He died of tumor progression on the 20th day after admission.

Conclusion: AT/RT is an additional category of brain tumors that cause the clinically and electro-physiologically critical condition in a few days after the onset.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clineuro.2021.106922DOI Listing

Publication Analysis

Top Keywords

atypical teratoid/rhabdoid
8
teratoid/rhabdoid tumor
8
background activity
8
tumor
6
acute-phase electroencephalography
4
electroencephalography infantile
4
infantile atypical
4
tumor background
4
background primary
4
primary brain
4

Similar Publications

Background: Atypical teratoid rhabdoid tumor (ATRT) is the most common malignant brain tumor in infants, and more than 60% of children with ATRT die from their tumor. ATRT is associated with mutational inactivation/deletion of , a member of the SWI/SNF chromatin remodeling complex, suggesting that epigenetic events play a critical role in tumor development and progression. Moreover, disruption of SWI/SNF allows unopposed activity of epigenetic repressors, which contribute to tumorigenicity.

View Article and Find Full Text PDF
Article Synopsis
  • Pediatric intracranial tumors at the skull base are rare, challenging to treat due to complex anatomy, and lack substantial clinical evidence, prompting a study on surgical approaches and outcomes.
  • The study involved 12 children under 18 who underwent skull base surgery from 2017 to 2023, analyzing demographics, tumor details, surgical methods, and survival rates.
  • Results indicated a predominance of female patients, varying tumor locations and types, with 58.3% achieving gross or near-total resection; however, 33.3% of patients died from tumor progression within an average of 15 months.
View Article and Find Full Text PDF

Atypical teratoid/rhabdoid tumors (AT/RTs) of the central nervous system (CNS) are rare and aggressive, typically occurring in early childhood or infancy, with adult cases being extremely rare. These tumors are associated with the inactivation of the integrase interactor 1 (INI1) gene. The prognosis is poor, worsening significantly if metastasis is detected at diagnosis.

View Article and Find Full Text PDF

Designing a Time-Dependent Therapeutic Strategy using CDK4/6 Inhibitors in an Intracranial ATRT Model.

Neuro Oncol

December 2024

Department of Neurological Surgery, Weill Cornell Medicine, New York, NY 10065, USA.

Background: Inhibitors targeting cyclin-dependent kinases 4 and 6 (CDK4/6), crucial for cell cycle regulation, have shown promise in early-stage studies for treating central nervous system (CNS) tumors. However, challenges such as limited CNS penetration, optimal treatment duration, and systemic side effects have impeded their clinical translation for pediatric brain tumors (PBTs).

Methods: We evaluated the potency of CDK4/6 inhibitors across various PBTs cell lines, focusing particularly on palbociclib against atypical teratoid rhabdoid tumor (ATRT) with cell viability assays and gene expression analysis.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!