Potential Late Effects of Rhabdoid Tumor Therapy in Childhood and Adolescents

Author(s):  
Karolina Nemes ◽  
Michael C. Frühwald
2004 ◽  
Vol 22 (14) ◽  
pp. 2877-2884 ◽  
Author(s):  
Joanne M. Hilden ◽  
Sharon Meerbaum ◽  
Peter Burger ◽  
Jonathan Finlay ◽  
Anna Janss ◽  
...  

Purpose Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines. A registry has been established to create an outcomes database and to facilitate biology studies for this tumor. Materials and Methods A standardized data sheet was provided to treating physicians listing the reports that were to be sent to the registry for abstraction. Follow-up information was sought twice yearly. Results Information was complete for 42 patients. Median age at diagnosis was 24 months. Nine patients (21%) had disseminated disease at diagnosis. Sixteen tumors were infratentorial; 26 were supratentorial. Twenty patients (48%) received a primary complete resection. Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), stem-cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%). Recurrent or progressive disease was reported in nine and 19 patients, respectively. Twenty-seven patients (64%) are dead of disease (3 to 62 months from diagnosis) and one patient died of toxicity. Fourteen patients (33%) show no evidence of disease (9.5 to 96 months from diagnosis). The median survival is 16.75 months and the median event-free survival is 10 months. Conclusion Aggressive therapy has prolonged the natural history in a subset of children. Prospective multi-institutional and national clinical trials designed specifically for AT/RT are needed. Enrollment onto the AT/RT registry should be continued.


Author(s):  
Minh Nguyen ◽  
Guang Huan-Tu ◽  
Melissa Gonzalez-Edick ◽  
Victor M Rivera ◽  
Tim Clackson ◽  
...  
Keyword(s):  

1984 ◽  
Vol 23 (02) ◽  
pp. 87-91 ◽  
Author(s):  
K. Flemming

SummaryIn the beginning of medical radiology, only the benefit of ionizing radiation was obvious, and radiation was handled and applied generously. After late effects had become known, the radiation exposure was reduced to doses following which no such effects were found. Thus, it was assumed that one could obtain an optimal medical benefit without inducing any hazard. Later, due to experimental findings, hypotheses arose (linear dose-effect response, no time factor) which led to the opinion that even low and lowest radiation doses were relevant for the induction of late effects. A radiation fear grew, which was unintentionally strengthened by radiation protection decrees: even for low doses a radiation risk could be calculated. Therefore, it was believed that there could still exist a radiation hazard, and the radiation benefit remained in question. If, however, all presently known facts are considered, one must conclude that large radiation doses are hazardous and low doses are inefficient, whereas lowest doses have a biopositive effect. Ionizing radiation, therefore, may cause both, hazard as well as benefit. Which of the two effects prevails is determined by the level of dose.


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