Atypical Teratoid/Rhabdoid Tumors

Part of the Pediatric Cancer book series (PECA, volume 3)


Atypical teratoid/rhabdoid tumor is a rare aggressive malignant central nervous system tumor that most often occurs in young children. Though these tumors may have overlapping histological features with other Central Nervous System (CNS) tumors, one feature unique to most atypical teratoid/rhabdoid tumors is a genetic abnormality in the INI-1 gene on chromosome 22q11. Loss of the INI-1 protein staining is important for distinguishing atypical teratoid/rhabdoid tumors from other tumors. Historically, outcomes for patients with atypical teratoid/rhabdoid tumors have been poor despite surgery and chemotherapy. Promising new strategies, including intensive multimodal therapy and high dose chemotherapy with autologous stem cell transplantation, have significantly improved outcomes. In this chapter, we present a comprehensive overview of atypical teratoid/rhabdoid tumors. We focus upon their epidemio­logy, molecular genetics, pathologic diagnosis and clinical presentation. In addition, we discuss treatment approaches, prognostic factors, outcomes and future research directions.


Overall Survival Autologous Stem Cell Transplantation Central Nervous System Tumor Rhabdoid Tumor Choroid Plexus Carcinoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  1. 1.Neurosurgery Research ProgramChildren’s Hospital of Chicago Research Center and Northwestern University Feinberg School of MedicineChicagoUSA
  2. 2.Hematology, Oncology and Stem Cell TransplantationAnn & Robert H. Lurie Children’s Hospital of Chicago and Northwestern University Feinberg School of MedicineChicagoUSA

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