Pediatric Atypical Teratoid/Rhabdoid Tumors: Dissemination to the Cerebrospinal Fluid

  • Junjeong Choi
  • Hyosun Kim
  • Se Hoon Kim
Part of the Pediatric Cancer book series (PECA, volume 3)


About a third of the patients of atypical teratoid rhabdoid tumor (AT/RT) present with positive CSF cytology at the time of first diagnosis and spinal and leptomeningeal spread ultimately develops in more than a half of the patients regardless of the local recurrence of the disease. Thus, CSF cytology is clinically important in terms of the assessment of the disease and the determination of treatment modalities as well as monitoring the course of the disease and the response to the therapy. Most of the reported AT/RT cases described large atypical cells, abundant cytoplasm, eccentrically placed nuclei, and prominent nucleoli with cellular clustering in the CSF cytology. A potential association of E-cadherin in the cellular clustering in AT/RT was suggested with comparison of medulloblastoma cases. Chang’s M staging has been used for AT/RT staging, but it is likely that the application of this system to all AT/RTs in diverse anatomical location has limitations. Accordingly, a new staging system should be developed. Liquid based cytology preparation is satisfactory for the morphologic diagnosis of AT/RT in CSF.


Abundant Cytoplasm Cytospin Preparation Atypical Teratoid Rhabdoid Tumor Leptomeningeal Dissemination Liquid Base Cytology 
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Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  1. 1.Department of PathologyYonsei University Wonju College of MedicineWonjuRepublic of Korea
  2. 2.Department of PediatricsYonsei University College of MedicineSeoulRepublic of Korea
  3. 3.Department of PathologyYonsei University College of MedicineSeoulRepublic of Korea

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