Assessment of the treatment approach and survival outcomes in a modern cohort of patients with atypical teratoid rhabdoid tumors using the National Cancer Database

Abstract

Background: Primary gliosarcoma is a rare malignant brain tumor with dismal prognosis. Previous reports are limited to case reports and small retrospective case series. Objective: To evaluate treatment and survival outcomes in a large cohort of primary gliosarcoma patients treated in the United States. Results: 1622 patients met the inclusion criterion. Median age was 63 years. The 3-year OS rate for the entire cohort was 11.9%. Patients aged 18 to 60 years were significantly more likely to receive trimodality therapy (defined as the use of surgery, radiotherapy [RT] and chemotherapy [CT]) than patients older than 60 (68.1% vs. 56.7%, p < 0.001). The utilization of trimodality therapy significantly increased during the study period (57.5% in 2004-2008 vs. 65.1% in 2009-2013; p = 0.002). On multivariate Cox regression analysis, GTR, surgery followed by RT and the use of trimodality therapy were associated with longer OS, while older age, Charlson-Deyo score ≥ 1 and multi-focal tumor were associated with shorter OS. The use of trimodality therapy was consistently associated with longer OS in subgroup analyses based on age and extent of resection. Materials and Methods: The National Cancer Database was used to identify all primary gliosarcoma patients aged 18 to 90 years who were diagnosed between 2004 and 2013. Overall survival (OS) was evaluated by Kaplan-Meir analysis, univariate and multivariate Cox proportional hazard regression analysis. Conclusions: The use of trimodality therapy significantly increased during the study period and was associated with improved outcomes regardless of age and extent of resection.

MDM2 and MDM4 Are Therapeutic Vulnerabilities in Malignant Rhabdoid Tumors

“…For efficacy studies, TTC642 cells were injected into the right flank as above. When tumors reached approximately 150-250 mm 3 , mice were randomized [stratified randomization; Study Director 3.1 (Studylog)] to vehicle, idasanutlin, or ATSP-7041 (n ¼ 8/arm). Vehicle and idasanutlin were dosed at 150 mg/kg orally twice per day for 5 days.…”

“…Malignant rhabdoid tumors (MRT) are highly aggressive cancers associated with extremely poor prognoses despite intensive therapy (1)(2)(3). MRTs typically arise in young children in the kidney, brain (atypical teratoid/rhabdoid tumors, ATRT), and soft tissues (4).…”

Trimodality therapy for atypical teratoid/rhabdoid tumor is associated with improved overall survival: A surveillance, epidemiology, and end results analysis

“…This is largely due to the observation of heightened late CNS toxicity as a function of dose and younger age at the time of irradiation; specifically, intellectual impairment, endocrine dysfunction, growth disturbances, and increased secondary malignancy have been reported . Nevertheless, the clinical benefit of RT in the management of AT/RT has been published in a number of series; but, in one of the largest series, <40% of patients received RT as part of their management . The purpose of the current investigation is to robustly analyze the impact of RT among pediatric AT/RT patients who underwent definitive surgical resection followed by receiving chemotherapy using the Surveillance, Epidemiology, and End Results (SEER) database.…”

“…[15][16][17] Nevertheless, the clinical benefit of RT in the management of AT/RT has been published in a number of series [2][3][4][18][19][20][21][22][23][24] ; but, in one of the largest series, <40% of patients received RT as part of their management. 25 The purpose of the current investigation is to robustly analyze the impact of RT among pediatric AT/RT patients who underwent definitive surgical resection followed by receiving chemotherapy using the Surveillance, Epidemiology, and…”

miR-16 Suppresses Growth of Rhabdoid Tumor Cells

“…In the brain, it is known as "atypical teratoid/rhabdoid tumor" (ATRT) 1 . Despite the use of intensive chemotherapy regimens, including high-dose chemotherapy with autologous stem cell rescue, prognosis remains poor regardless of anatomic site [1][2][3][4] . Among renal tumors, rhabdoid tumor was recognized as a separate entity from Wilms tumor in 1981 5 .…”