An 18-year-old woman with malignant teratoid medulloepithelioma had an epibulbar, brown-black mass associated with expulsive hemorrhage and a limboscleral perforation. Histopathologically, a prominent pigmented neuroepithelial component was present and correlated to the black color of the mass. The massive hemorrhage is apparently related to rupture of necrotic and abnormal vascular channels within the angiomatous component of the neoplasm. Other mesenchymal elements included spindle-shaped cells, rhabdomyoblasts, and islands of hyaline cartilage.
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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 PDFAtypical 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.
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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).
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