BACKGROUND: Malignant rhabdoid tumors (MRTs) are aggressive and often fatal; the Surveillance, Epidemiology, and End Results (SEER) database offers an opportunity to study this rare malignancy. METHODS: From the SEER database, we extracted records of patients with a reported diagnosis of MRT and analyzed them for clinical features and survival rates by univariate and multivariate analyses. RESULTS: For the 229 patients included in our data, who were diagnosed from 1986 to 2005, primary tumors were located in the central nervous system (CNS) (35%), kidneys (20%), and extra-renal non-cranial sites (ERNC-MRTs) (45%). Most patients with renal and CNS tumors were less than 18 years old (87% and 96%, respectively) while more than half of the patients with ERNC-MRTs (61%) were adults. Among staged tumors, 23% were localized, 34% regional, and 43% distant. Renal tumors had significantly more metastatic disease (47%; P = 0.006) than ERNC-MRTs. The estimated 5-year survival for the entire group was 33 +/- 3.4% (SE). Univariate and multivariate analyses showed that age at diagnosis (2-18 years), localized stage of tumors, and use of radiotherapy were significantly associated with improved survival. Adults had a better outcome than young children (<2 years old) but a poorer outcome than older children (2-18 years old); tumor stage, but not radiotherapy use, affected outcome in adults. The survival and prognostic factors of children diagnosed before and after 2000 did not differ significantly. CONCLUSION: Our population-based study indicates that age at diagnosis, tumor stage, and use of radiotherapy favorably impact survival rates of patients with MRTs. Copyright 2009 Wiley-Liss, Inc.
BACKGROUND:Malignant rhabdoid tumors (MRTs) are aggressive and often fatal; the Surveillance, Epidemiology, and End Results (SEER) database offers an opportunity to study this rare malignancy. METHODS: From the SEER database, we extracted records of patients with a reported diagnosis of MRT and analyzed them for clinical features and survival rates by univariate and multivariate analyses. RESULTS: For the 229 patients included in our data, who were diagnosed from 1986 to 2005, primary tumors were located in the central nervous system (CNS) (35%), kidneys (20%), and extra-renal non-cranial sites (ERNC-MRTs) (45%). Most patients with renal and CNS tumors were less than 18 years old (87% and 96%, respectively) while more than half of the patients with ERNC-MRTs (61%) were adults. Among staged tumors, 23% were localized, 34% regional, and 43% distant. Renal tumors had significantly more metastatic disease (47%; P = 0.006) than ERNC-MRTs. The estimated 5-year survival for the entire group was 33 +/- 3.4% (SE). Univariate and multivariate analyses showed that age at diagnosis (2-18 years), localized stage of tumors, and use of radiotherapy were significantly associated with improved survival. Adults had a better outcome than young children (<2 years old) but a poorer outcome than older children (2-18 years old); tumor stage, but not radiotherapy use, affected outcome in adults. The survival and prognostic factors of children diagnosed before and after 2000 did not differ significantly. CONCLUSION: Our population-based study indicates that age at diagnosis, tumor stage, and use of radiotherapy favorably impact survival rates of patients with MRTs. Copyright 2009 Wiley-Liss, Inc.
Authors: Diane K Birks; Andrew M Donson; Purvi R Patel; Christopher Dunham; Andrea Muscat; Elizabeth M Algar; David M Ashley; B K Kleinschmidt-Demasters; Rajeev Vibhakar; Michael H Handler; Nicholas K Foreman Journal: Neuro Oncol Date: 2011-09-23 Impact factor: 12.300
Authors: Julia E Heck; Christina A Lombardi; Myles Cockburn; Travis J Meyers; Michelle Wilhelm; Beate Ritz Journal: Pediatr Blood Cancer Date: 2012-03-20 Impact factor: 3.167
Authors: D Callegaro-Filho; D M Gershenson; A M Nick; M F Munsell; P T Ramirez; P J Eifel; E D Euscher; R M Marques; S M Nicolau; K M Schmeler Journal: Gynecol Oncol Date: 2015-11-04 Impact factor: 5.482