Atypical Teratoid Rhabdoid Tumor
Atypical teratoid/rhabdoid tumors are malignant high grade embryonal (WHO Grade IV) tumors seen in children often below the age of 3 years and rarely above the age of 6 years. Account for 10% of tumors in infants with slight male predominance, M:F ratio = 2:1 Characteristically poorly differentiated, contain rhabdoid cells with divergent differentiation to form epithelial, mesenchymal, neuronal, and glial components and may be associated with a subpopulation of primitive neuroectodermal cells.
KeywordsAtypical teratoid rhabdoid tumor PNET Embryonal tumor Rhabdoid predisposition syndrome
- Burger PC, Yu IT, Tihan T, Friedman HS, Strother DR, Kepner JL, Duffner PK, Kun LE, Perlman EJ (1998) Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma. Am J Surg Pathol 22:1083–1092PubMedCrossRefGoogle Scholar
- Judkins AR, Burger PC, Hamilton RL, Kleinschmidt-DeMasters B, Perry A, Pomeroy SL, Rosenblum MK, Yachnis A, Zhou H, Rorke LB, Biegel JA (2005) INI 1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neuropathol Exp Neurol 64:391–397PubMedGoogle Scholar
- Jackson EM, Shaikh TH, Gururangan S, Jones MC, Malkin D, Nikkel SM, Zuppan CW, Wainwright LM, Zhang F, Biegel JA (2007) High-density single nucleotide polymorphism array analysis in patients with germline deletions of 22q11.2 and malignant rhabdoid tumor. Hum Genet 122(2):117–27PubMedCrossRefGoogle Scholar