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Sarcoma  2013 

Surgery and Actinomycin Improve Survival in Malignant Rhabdoid Tumor

DOI: 10.1155/2013/315170

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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. 1. Introduction Malignant rhabdoid tumor (MRT) was first described as a rhabdomyosarcomatous subtype of Wilms Tumor in 1978 [1] and recognized as a distinct entity in 1981 [2]. Malignant rhabdoid tumors most commonly occur in children with extrarenal variants seen in the CNS, liver, female genital tract, and soft tissues. Extrarenal malignant rhabdoid tumor is a rare tumor with a poor prognosis. The medical literature is replete with papers describing the highly lethal nature of MRT. We report on demographics and treatment variables as prognostic after compiling data from the literature on extrarenal extra-CNS MRT. 2. Methods 2.1. Eligibility and Search Strategy A search was conducted for pure primary extrarenal extracentral nervous system malignant rhabdoid tumors. Ovid Medline was searched for articles containing “rhabdoid tumor” or listed under the MeSH “rhabdoid tumor” in English. This search was conducted between the years of 1981 and April 2006 as MRT was not recognized as a distinct entity until 1981. As this is a study of extrarenal extracentral nervous system MRT cases of paraspinal tumors were excluded. Cases described as carcinomas or other primary tumor types with “rhabdoid features” or a “rhabdoid

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