title: Atypical teratoid/rhabdoid tumors: the need for more active therapeutic measures in younger patients.
abstract: Despite active and aggressive therapeutic measures, atypical teratoid/rhabdoid tumors (AT/RTs) are without doubt one of the most malignant brain tumors that occur during childhood. These tumors are more prevalent in children less than 3 years of age, and the prognosis is worse in these young children. Medical records of 14 patients diagnosed at our institution were reviewed. Patients diagnosed with AT/RT at our institution between 1997 and 2009 were identified. Clinical data for those patients with pathologic confirmation of AT/RTs were reviewed regarding surgery, adjuvant treatment, and survival. A total of 14 patients were identified. One patient who died 3 days after initial surgery was excluded, and 13 patients were recruited. Of the 13 patients, 7 were less than 1 year of age, and only 2 were over 3 years of age. Nine patients showed dissemination at diagnosis. Four patients were treated with radiation therapy (RTx), and 2 of these were less than 3 years of age. Ten patients had chemotherapy (CTx), and 3 of these underwent high dose chemotherapy and autologous peripheral blood stem cell transplantation. The median progression-free survival (PFS) was 4 months, and the median overall survival (OS) was 9 months. Univariable analysis revealed age more than 1 year, CTx, and RTx to be significant factors for better PFS and OS. None of the variables were significant in multivariable analysis. Young patients with AT/RT have a bad prognosis. An aggressive therapeutic strategy needs to be profiled for this age group.
authors: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 110-744, Republic of Korea.
authors: Ji Yeoun
authors: JY
authors: Lee
authors: In-Kyeong
authors: IK
authors: Kim
authors: Ji Hoon
authors: JH
authors: Phi
authors: Kyu-Chang
authors: KC
authors: Wang
authors: Byung-Kyu
authors: BK
authors: Cho
authors: Sung-Hye
authors: SH
authors: Park
authors: Hyo Seop
authors: HS
authors: Ahn
authors: Il Han
authors: IH
authors: Kim
authors: Seung-Ki
authors: SK
authors: Kim
mesh_terms: D001932:Brain Neoplasms / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007223:Infant; D008297:Male; D009004:Monosaccharide Transport Proteins / Q000378:metabolism; D011379:Prognosis; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000401:mortality / Q000601:surgery*; D016019:Survival Analysis; D013724:Teratoma / Q000401:mortality / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D014746:Vimentin / Q000378:metabolism
chemical_list: D005904:Glial Fibrillary Acidic Protein; D009004:Monosaccharide Transport Proteins; D014746:Vimentin