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Sarcoma
Volume 2013 (2013), Article ID 315170, 8 pages
http://dx.doi.org/10.1155/2013/315170
Research Article

Surgery and Actinomycin Improve Survival in Malignant Rhabdoid Tumor

1Department of Orthopaedic Surgery, University of Minnesota, 2450 Riverside Avenue South R200, Minneapolis, MN 55454, USA
2Department of Laboratory Medicine and Pathology, University of Minnesota, 2450 Riverside Avenue South R200, Minneapolis, MN 55454, USA

Received 5 September 2012; Accepted 1 January 2013

Academic Editor: R. Pollock

Copyright © 2013 Ryan Horazdovsky et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose. Malignant rhabdoid tumor (MRT) is an uncommon tumor that rarely occurs outside of renal and central nervous system (CNS) sites. Data from the literature were compiled to determine prognostic factors, including both demographic and treatment variables of malignant rhabdoid tumor, focusing on those tumors arising in extra-renal, extra-CNS (ER/EC MRT) sites. Patients and Methods. A systematic review and meta-analysis was performed by extracting demographic, treatment, and survival follow up on 167 cases of primary ER/EC MRT identified in the literature. Results. No survival differences were observed between those treated with or without radiation, or with or without chemotherapy. A Cox regression of overall survival revealed several independent prognostic factors. Surgical excision had a 74% (P = 0.0003) improvement in survival. Actinomycin had a 73% (P = 0.093) improvement in survival. Older age was associated with improved survival. The four-year survival, by Kaplan-Meier estimates, comparing patients less than two years old versus older than two at diagnosis was 11% versus 35%, respectively (P = 0.0001, Log-Rank). Conclusion. ER/EC MRT is a rare, soft-tissue tumor with a poor prognosis most commonly occurring in children. Surgical resection, treatment with actinomycin, and older age at diagnosis are all associated with improved survival.