Pediatric Atypical Teratoid/Rhabdoid Tumors: Differential Diagnosis

  • Justin A. Bishop
  • Syed Z. Ali
Part of the Pediatric Cancer book series (PECA, volume 2)


Atypical teratoid/rhabdoid tumor (AT/RT) is a rare malignancy of the ­central nervous system. It occurs most often in the posterior cranial fossa of children younger than 2 years old. The microscopic appearance of AT/RT is quite variable. Though rhabdoid cells are characteristic of AT/RT, they are often dominated by other cell populations. Owing to its varying morphologies, many entities should be considered in the differential diagnosis of AT/RT. These tumors include medulloblastoma, choroid plexus carcinoma, glioblastoma, and germ cell tumors. Deletions or mutations on the long arm of chromosome 22 resulting in decreased expression of INI1 are seen in nearly all cases of AT/RT. The recent introduction of an immunohistochemical stain for INI1 has greatly aided pathologists in distinguishing AT/RT from other morphological mimics.


Glial Fibrillary Acidic Protein Posterior Fossa Germ Cell Tumor Epithelioid Sarcoma Choroid Plexus Carcinoma 
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Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  1. 1.Department of PathologyThe Johns Hopkins HospitalBaltimoreUSA
  2. 2.Department of Pathology and RadiologyThe Johns Hopkins HospitalBaltimoreUSA

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