Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs)

• Published in: Pediatric blood & cancer Vol. 54; no. 4; pp. 647 - 648
• Main Authors: Garrè, Maria Luisa, Tekautz, Tanya
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.04.2010
• Subjects: Antineoplastic Agents - therapeutic use; AT/RT; Central Nervous System Neoplasms - drug therapy; Clinical Trials as Topic; CNS; high-dose chemotherapy; Humans; Infant; Infant, Newborn; Rhabdoid Tumor - drug therapy; Teratoma - drug therapy
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.22377

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.