Journal
PEDIATRIC BLOOD & CANCER
Volume 61, Issue 5, Pages 919-921Publisher
WILEY-BLACKWELL
DOI: 10.1002/pbc.24793
Keywords
atypical teratoid; rhabdoid tumor; SMARCB1 germline mutation; treatment outcome
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Funding
- Fordergemeinschaft Kinder-Krebs-Zentrum, Hamburg
- Deutsche Kinderkrebsstiftung
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Rhabdoid tumor predisposition syndrome is usually associated with shorter survival in patients with malignant rhabdoid tumors regardless of anatomical origin. Here we present four children harboring truncating heterozygous SMARCB1/INI1 germline mutations with favorable outcome. All four patients received multi-modality treatment, three according to therapeutic recommendations by the EU-RHAB registry, two without radiotherapy, and mean event-free survival accounts for 7 years. In conclusion, intensive treatment with curative intent is justified for children with rhabdoid tumors even if an underlying rhabdoid predisposition syndrome is demonstrated. Pediatr Blood Cancer 2014;61:919-921. (c) 2013 Wiley Periodicals, Inc.
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