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Malignant Rhabdoid Tlimor of Brain: An Aggressive Clinical Entity

Published online by Cambridge University Press:  18 September 2015

B.J. Fisher ,
J. Siddiqui ,
D. MacDonald ,
A.E. Cairney ,
D. Ramsey ,
D. Munoz  and
R. Del Maestro
B.J. Fisher*
Affiliation:
Department of Radiation Oncology, London Regional Cancer Centre, London
J. Siddiqui
Affiliation:
Department of Neurosurgery, London Health Sciences Centre, London
D. MacDonald
Affiliation:
Department of Neurology, London Health Sciences Centre, London
A.E. Cairney
Affiliation:
Department of Paediatrics, London Health Sciences Centre, London
D. Ramsey
Affiliation:
Department of Pathology, London Health Sciences Centre, London
D. Munoz
Affiliation:
Department of Pathology, London Health Sciences Centre, London
R. Del Maestro
Affiliation:
Department of Neurosurgery, London Health Sciences Centre, London
*
London Regional Cancer Centre, 790 Commissioners Road East, London, Ontario, Canada N6A 4L6
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Abstract

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Objective: We report three patients with malignant rhabdoid tumor (MRT) of the brain, two children and an adult. There were three purposes to this report: to describe the clinical course in an adult with MRT; to describe the interesting histopathological metamorphosis of one of the tumors; and to report the outcome of the treatment regimens we used in order to help guide future treatment. Since these tumors are quite rare it is important to continue to try new regimens in the search for effective therapy rather than to repeat ineffective ones. Method: Report of three patients. Results: The clinical course in all three patients was typical of these aggressive neoplasms in that chemotherapy and radiotherapy were ineffective in modifying the rapid deterioration leading to death. Conclusions: MRT can occur in adults. Autopsy in one patient showed that the tumor seemed to undergo an evolution in appearance when compared with the original pathology specimen from craniotomy. Administration of systemic therapy should be prompt and include intrathecal chemotherapy.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 23 , Issue 4 , November 1996 , pp. 257 - 263
Copyright
Copyright © Canadian Neurological Sciences Federation 1996

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