Clinical and pathological features of paediatric malignant rhabdoid tumours
Article first published online: 3 AUG 2009
Copyright © 2009 Wiley-Liss, Inc.
Pediatric Blood & Cancer
Volume 54, Issue 1, pages 29–34, January 2010
How to Cite
Morgenstern, D. A., Gibson, S., Brown, T., Sebire, N. J. and Anderson, J. (2010), Clinical and pathological features of paediatric malignant rhabdoid tumours. Pediatr. Blood Cancer, 54: 29–34. doi: 10.1002/pbc.22231
- Issue published online: 9 NOV 2009
- Article first published online: 3 AUG 2009
- Manuscript Accepted: 8 JUL 2009
- Manuscript Received: 15 JUN 2009
Malignant rhabdoid tumours (MRT) and their central nervous system (CNS) counterparts atypical teratoid/rhabdoid tumours (ATRT) are rare, highly aggressive malignant neoplasms of childhood. Although there are isolated reports of long-term survival with intensive, multimodal therapy, outcomes are generally poor.
We conducted a retrospective review of all patients diagnosed with MRT/ATRT at Great Ormond Street Hospital over the 20 years from 1989 to 2009. All cases were subjected to expert pathological review including INI-1 immunostaining.
In a final cohort of 34 cases, overall survival was 17.4%, with median survival 10.1 months. Outcome in patients aged <3 years was significantly worse (median survival 6.2 months vs. 19.2 months). Data demonstrated a statistically significant benefit of radiotherapy (median survival 14.9 months vs. 6.6 months), although this analysis is confounded by the impact of patient age. There were four long-term survivors (>30 months), all of whom received chemotherapy with or without surgical resection or radiotherapy. In the present study, immunohistochemistry revealed no significant staining for either c-Erb or c-Met in any case, suggesting that targeting these molecules is unlikely to be of benefit in treating MRT/ATRT.
In view of poor outcomes, there is a clear need for new treatment strategies and the identification of novel molecular targets for MRT/ATRT. Pediatr Blood Cancer 2010; 54:29–34. © 2009 Wiley-Liss, Inc.