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Atypical Teratoid/Rhabdoid Tumor (AT/RT)
Axial Noncontrast Head CT and T2 MRI of a AT/RT in the cerebellopontine angle (CPA), which causes CSF outflow obstruction and obstructive hydrocephalus.
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Atypical Teratoid/Rhabdoid Tumor (AT/RT)

Overview
    • Malignant, high-grade (Grade 4), pediatric-onset embryonal tumor.
Clinical Presentation
  • Pediatric tumor: onset typically < 3 y.o.
  • Most common location:
    • Cerebellopontine angle (Obstructive hydrocephalus) - (majority will have < 2 yr survival)
  • Less common location:
    • Cerebrum - (if localized and with favorable immunohistochemical characteristics will have ~ 5 yr survival)
    • Suprasellar region (CN deficits)
    • Pineal region
  • Rare location:
    • Spinal cord
  • Leptomeningeal/CSF dissemination is common and a worrisome (often terminal) finding.
Pathology
  • Hemorrhagic/necrotic tumors that comprise sheets of poorly differentiated rhabdoid cells.
    • Cells have hyperchromatic nuclei, scant cytoplasm, and frequent mitoses.
  • Contain components of divergent cellular differentiaion:
    • Epithelial
    • Mesenchymal (produces a firm-texture)
    • Neuronal
    • Glial
Immunohistochemistry
  • Immunopositivity for the following:
    • Epithelial membrane antigen (EMA)
    • Vimentin
    • +/- Smooth muscle actin (SMA)
Differential Diagnosis
References
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  • Adesina, Adekunle M., Tarik Tihan, Christine E. Fuller, and Tina Young Poussaint. Atlas of Pediatric Brain Tumors. Springer, 2016.
  • Chakrabarti, Indranil, Kaushik Majumdar, and Amita Giri. “Infratentorial Medulloepithelioma with Divergent Differentiation: Possibly a Predictor of Poor Outcome.” Journal of Pediatric Neurosciences 7, no. 2 (2012): 142–45. https://doi.org/10.4103/1817-1745.102581.
  • “Childhood Central Nervous System Embryonal Tumors.” PdqCancerInfoSummary. National Cancer Institute. Accessed June 14, 2018. https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq.
  • Gupta, Anshu, and Tanima Dwivedi. “A Simplified Overview of World Health Organization Classification Update of Central Nervous System Tumors 2016.” Journal of Neurosciences in Rural Practice 8, no. 4 (2017): 629–41. https://doi.org/10.4103/jnrp.jnrp_168_17.
  • Holcomb, George W., Jerry D. Murphy, and Daniel J. Ostlie. Ashcraft’s Pediatric Surgery E-Book. Elsevier Health Sciences, 2014.
  • Institute, Dr Maria Tsokos, National Cancer. Shown Is a Microscopic View of a Typical Neuroblastoma with Rosette Formation. Magnified X40. December 1993. http://visualsonline.cancer.gov/details.cfm?imageid=2593. https://commons.wikimedia.org/wiki/File:Neuroblastoma_rosettes.jpg.
  • Jensflorian. English: Histology of Anaplastic Medulloblastoma with Increased Mitotic Activity and Cell Pleomorphim. October 11, 2010. Own work. https://commons.wikimedia.org/wiki/File:Anaplastic_medulloblastoma_x200.jpg.
  • Louis, David N., Arie Perry, Guido Reifenberger, Andreas von Deimling, Dominique Figarella-Branger, Webster K. Cavenee, Hiroko Ohgaki, Otmar D. Wiestler, Paul Kleihues, and David W. Ellison. “The 2016 World Health Organization Classification of Tumors of the Central Nervous System: A Summary.” Acta Neuropathologica 131, no. 6 (June 1, 2016): 803–20. https://doi.org/10.1007/s00401-016-1545-1.
  • “Meeting Library | New Classification for Central Nervous System Tumors: Implications for Diagnosis and Therapy.” Accessed May 31, 2018. https://meetinglibrary.asco.org/record/138232/edbook#fulltext.
  • Newton, Herbert B. Handbook of Brain Tumor Chemotherapy, Molecular Therapeutics, and Immunotherapy. Academic Press, 2018.
  • Orkin, Stuart H., David E. Fisher, A. Thomas Look, Samuel Lux, David Ginsburg, and David G. Nathan. Oncology of Infancy and Childhood E-Book. Elsevier Health Sciences, 2009.
  • Perry, Arie. “WHO’s Arrived in 2016! An Updated Weather Forecast for Integrated Brain Tumor Diagnosis.” Brain Tumor Pathology 33, no. 3 (July 1, 2016): 157–60. https://doi.org/10.1007/s10014-016-0266-4.
  • Prayson, Richard A., and Mark L. Cohen. Practical Differential Diagnosis in Surgical Neuropathology. Springer Science & Business Media, 2000.
  • Reni, Michele, Elena Mazza, Silvia Zanon, Gemma Gatta, and Charles J. Vecht. “Central Nervous System Gliomas.” Critical Reviews in Oncology/Hematology 113 (May 2017): 213–34. https://doi.org/10.1016/j.critrevonc.2017.03.021.
  • Ryall, Scott, Uri Tabori, and Cynthia Hawkins. “A Comprehensive Review of Paediatric Low-Grade Diffuse Glioma: Pathology, Molecular Genetics and Treatment.” Brain Tumor Pathology 34, no. 2 (April 1, 2017): 51–61. https://doi.org/10.1007/s10014-017-0282-z.
  • Wippold, F. J., and A. Perry. “Neuropathology for the Neuroradiologist: Rosettes and Pseudorosettes.” American Journal of Neuroradiology 27, no. 3 (March 1, 2006): 488–92.

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