Embryonal Tumors

Pediatric-onset tumors that originate from embryonic cells that persist after birth

• Medulloblastoma, G4
• Malignant, embryonal tumor of the cerebellum.
• Sporadic, typically, but infrequently will occur with certain hereditary syndromes (see below).
• Accounts for 20% of malignant CNS pediatric tumors.
• Embryonal tumor with multilayered rosettes (C19MC-altered), G4
• Hisopathology: ependymoblastmatous multilayered rosettes.
• Medulloepithelioma, G4
• Hisopathology: neuroepithelium reminiscent of the embryonic neural tube.
• CNS embryonal tumor, NOS, G4
• Atypical teratoid/rhabdoid tumor (AT/RT), G4
• Malignant, high-grade embryonal tumors.
• Hemorrhagic/necrotic tumors with sheets of poorly differentiated rhabdoid cells and divergent differentiaion: epithelial, mesenchymal (producing a firm-texture), neuronal, and gial components.
• Varying locations: supratentorial, infratentorial, or spinal cord (rarely).
• CNS embryonal tumor with rhabdoid features, G4

• Although this term is widespread in the literature, the term primitive neuroectodermal tumor (PNET) has been removed from the 2016 WHO diagnostic lexicon but refers to embryonal tumors of the central nervous system that localize to the cerebrum (rather than the cerebellum).

• “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.

• 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.

• 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.