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- Title
Atypical teratoid/rhabdoid tumors: the need for more active therapeutic measures in younger patients.
- Authors
Lee, Ji; Kim, In-Kyeong; Phi, Ji; Wang, Kyu-Chang; Cho, Byung-Kyu; Park, Sung-Hye; Ahn, Hyo; Kim, Il; Kim, Seung-Ki
- 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.
- Subjects
TERATOMA; BRAIN tumors; RADIOTHERAPY; STEM cell transplantation; ADJUVANT treatment of cancer; MEDICAL records; MULTIVARIATE analysis
- Publication
Journal of Neuro-Oncology, 2012, Vol 107, Issue 2, p413
- ISSN
0167-594X
- Publication type
Article
- DOI
10.1007/s11060-011-0769-0