Teratoid-rhabdoid tumor, atypical

MRT (atypical teratoid/rhabdoid tumor) frequently demonstrates deletion and mutation of the hSNF5/INIl gene, with decreased or absent INIl protein expression. An antibody to INIl can be used to assess INIl loss, characterized by absence of immunoreactivity in contrast to functional INIl with positive immunoreactivity in normal tissues and other neoplasms." Like MRT of the kidney and extrarenal soft tissues, atypical teratoid/rhabdoid tumors of the central nervous system showed absence of nuclear staining for INIl in a recent study." 3i... 

Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood definition of an entity./Neurosurg. 1996 85 56-65. 

Burger PC, Yu IT, Tihan T, et al. Atypical teratoid/rhabdoid tumor of the central nervous system a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma a Pediatric Oncology Group study. Am J Surg Pathol. 1998 22 1083-1092. 

Gessi M, Giangaspero F, Pietsch T. Atypical teratoid/rhabdoid tumors and choroid plexus tumors when genetics surprise pathology. Brain Pathol. 2003 13 409-414. 

Ogino S, Cohen ML, Abdul-Karim FW. Atypical teratoid/ rhabdoid tumor of the CNS cytopathology and immunohistochemistry of insulin-like growth factor-II, insulin-like growth factor receptor type 1, cathepsin D, and Ki-67. Mod Pathol. 1999 12 379-385. 

Haberler C, Laggner U, Slave I, et al. Immunohistochemical analysis of INIl protein in malignant pediatric CNS tumors Lack of INIl in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. Am J Surg Pathol. 2006 30 1462-1468. 

Biegel JA. Molecular genetics of atypical teratoid/rhabdoid tumor. Neurosurg Focus. 2006 20 E11. 

Uno K, Takita J, Yokomori K, et al. Aberrations of the hSNF5/ INIl gene are restricted to malignant rhabdoid tumors or atypical teratoid/rhabdoid tumors in pediatric solid tumors. Genes Chromosomes Cancer. 2002 34 33-41. 

Bruch LA, Hill DA, Cai DX, et al. A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid/rhabdoid tumors from medulloblas-toma/central primitive neuroectodermal tumors. Hum Pathol. 2001 32 156-162. 

Atypical teratoid-rhabdoid tumor Like PNET more cytoplasm Vimentin (-r) GFAP (S) cytokeratin (S) EMA (S) synaptophysin (R) chromogranin (R) Cerebellum brain... 

Nishihira Y, Tan CF, Hirato J, et al. A case of congenital supratentorial tumor Atypical teratoid/rhabdoid tumor or primitive neuroectodermal tumor Neuropathol. 2007 27 551-555. 

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