Background: Atypical teratoid/rhabdoid tumors (ATRT) are uncommon malignancies of the central nervous system and are often difficult to distinguish radiographically and pathologically from other common tumors. We present the first case of sellar ATRT presenting with subarachnoid hemorrhage (SAH) and intraventricular hemorrhage (IVH).
Case Description: A 62-year-old woman, who had presented with symptoms of headache, diabetes insipidus, hypothyroidism, and seizures, was found to have a sellar tumor with hemorrhagic transformation. Surgical resection was performed. The pathological examination findings were consistent with ATRT. Despite early surgical intervention, she later died before starting craniospinal radiotherapy and chemotherapy.
Conclusion: To the best of our knowledge, although known to present with intratumoral hemorrhage, to date, no cases of sellar ATRT have presented with SAH or IVH have been reported. Considering our finding that ATRT can present with SAH and IVH, establishing the correct diagnosis using radiographic imaging, gender, pathological findings, and molecular markers is paramount for speedy treatment and management.
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http://dx.doi.org/10.1016/j.wneu.2018.10.198 | DOI Listing |
Brain Spine
November 2024
Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany.
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 PDFNeuro 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.
Brain Spine
November 2024
Department of Neurosurgery, General Hospital of Nikaia 'Agios Panteleimon', 18454, Athens, Greece.
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