Purpose: To report a case of intraocular medulloepithelioma, an embryonal tumour with extremely rare presentation in adults.
Method: The case of a 44-year-old man with intraocular malignant teratoid medulloepithelioma, primarily diagnosed as intraocular teratoma, is described and the literature on this subject is reviewed.
Results: The patient presented with progressive proptosis caused by a tumour in the left eyeball. He had a 28-year history of loss of vision in the left eye. Histopathological examination of the enucleated eye demonstrated an intraocular teratoma. No adjuvant treatment was given. Six months later the patient presented with massive progression in the left orbit and intracranial invasion. Cisplatin-based chemotherapy was administered, but discontinued after two cycles due to poor tolerance and lack of response. At subsequent pathology review, a final diagnosis of malignant teratoid medulloepithelioma was made. Salvage radiotherapy (60 Gy in 30 fractions) resulted in partial response of the intracranial lesion. However, the patient died 6 months later due to intracranial tumour progression.
Conclusion: Medulloepithelioma should be considered in the differential diagnosis of intraocular tumours in adults, especially in the case of coexisting, long-standing ocular symptoms. In some cases this disease is very aggressive.
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http://dx.doi.org/10.1111/j.1600-0420.2005.00586.x | DOI Listing |
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.
Genome Biol
December 2024
Department of Neurology and Interdisciplinary Neuro-Oncology, Hertie Institute for Clinical Brain Research, University Hospital Tübingen, Eberhard Karls University Tübingen, Tübingen, 72076, Germany.
Background: Atypical teratoid rhabdoid tumors (ATRT) are incurable high-grade pediatric brain tumors. Despite intensive research efforts, the prognosis for ATRT patients under currently established treatment protocols is poor. While novel therapeutic strategies are urgently needed, the generation of molecular-driven treatment concepts is a challenge mainly due to the absence of actionable genetic alterations.
View Article and Find Full Text PDFViruses
November 2024
Human Genome and Stem Cell Research Center (CEGH-CEL), Department of Genetics and Evolutionary Biology, Institute of Biosciences, University of São Paulo, Cidade Universitária, São Paulo 05508-090, SP, Brazil.
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