Supratentorial embryonal tumors are very rare malignant tumors of neuroectodermal origin, characterized by an aggressive clinical behavior. They occur prevalently in children. They have been sporadically described in adults and represent an even rarer occurrence in elderly patients, raising many issues on the diagnostic pitfalls and their appropriate management. We present an unusual case of embryonal tumor in a 62-year-old man who presented with speech disorder, and partial deficit of the left 3rd and 5th cranial nerves secondary to a left temporo-insular embryonal tumor: the clinico-radiological features, histopathological insights, therapeutic options and results are discussed along with a review of the most relevant literature, addressing the specific issue of differential diagnosis and the expected results in the elderly population.
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http://dx.doi.org/10.5137/1019-5149.JTN.21006-17.1 | DOI Listing |
J Ayub Med Coll Abbottabad
November 2024
Akbar Niazi Teaching Hospital, IMDC, Islamabad.
Brain Tumor Pathol
October 2024
Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Acta Neuropathol Commun
September 2024
Department of Pathology, Neuropathology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105, USA.
Neuroepithelial tumors with fusion of PLAGL1 or amplification of PLAGL1/PLAGL2 have recently been described often with ependymoma-like or embryonal histology respectively. To further evaluate emerging entities with PLAG-family genetic alterations, the histologic, molecular, clinical, and imaging features are described for 8 clinical cases encountered at St. Jude (EWSR1-PLAGL1 fusion n = 6; PLAGL1 amplification n = 1; PLAGL2 amplification n = 1).
View Article and Find Full Text PDFActa Neuropathol Commun
June 2024
Department of Neuropathology, GHU Paris - Psychiatry and Neuroscience, Sainte-Anne Hospital, 1, rue Cabanis, Paris, 75014, France.
A novel histomolecular tumor of the central nervous system (CNS), the "diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC)," has recently been identified, based on a distinct DNA methylation profile and has been added to the 2021 World Health Organization Classification of CNS Tumors. This glioneuronal tumor mainly affects the supratentorial area in children and recurrently presents with a monosomy of chromosome 14. Herein, we report the case of a DNA-methylation based diagnosis of DGONC having atypical features, such as pseudo-rosettes and the absence of a chromosome 14 monosomy, thus rendering its diagnosis very challenging.
View Article and Find Full Text PDFChilds Nerv Syst
August 2024
Section of Pediatric-Oncology, Department of Oncology, Aga Khan University Hospital Karachi, Stadium Road, Karsaz, Karachi, Pakistan.
Background: ETMR is a unique and highly malignant brain tumor mostly occurring in infants. This report provides a comprehensive overview of the clinical presentation, histological aspects, radiological features, and therapeutic options of ETMR. Being the first report on the co-occurrence of NF1 with ETMR, it highlight the challenges of managing a patient with complex medical conditions.
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