Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs).
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.
Garrè
Maria Luisa
ML
Neuro-Oncology Unit, Haemato-Oncology and Neurosurgery Department, Giannina Gaslini Children's Research Hospital, Genova, Italy. mluisagarre@ospedale-gaslini.ge.it
Tekautz
Tanya
T
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D016543:Central Nervous System Neoplasms / Q000188:drug therapy*; D002986:Clinical Trials as Topic; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D018335:Rhabdoid Tumor / Q000188:drug therapy*; D013724:Teratoma / Q000188:drug therapy*
D000970:Antineoplastic Agents