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Role of high‐dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs)
Author(s) -
Garrè Maria Luisa,
Tekautz Tanya
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22377
Subject(s) - medicine , atypical teratoid rhabdoid tumor , monosomy , chemotherapy , pathology , medulloblastoma , radiation therapy , immunohistochemistry , blood cancer , cancer , chromosome , karyotype , biology , gene , biochemistry
Atypical teratoid/rhabdoid tumors (AT/RTs) of the CNS have been recently characterized as a distinct clinicopathologic entity with an unusually poor prognosis and with the highest incidence in the first 2 years of life. It often arises in the posterior fossa and its distinctive immunohistochemical (negative stain for INI‐1) and cytogenetic features (monosomy or deletion of chromosome 22) permit an adequate diagnosis in most of cases. AT/RT of the CNS is a usually fatal disease virtually unresponsive to chemotherapy (CT) and radiotherapy (RT). Rapid progression and CNS dissemination are commonly reported. Whether combined regimens including high‐dose CT are able to prolong survival or change the natural history of this tumor are under evaluation. Pediatr Blood Cancer 2010;54:647–648. © 2010 Wiley‐Liss, Inc.