AI Article Synopsis
- Atypical teratoid rhabdoid tumors (AT/RT) are aggressive central nervous system tumors mostly affecting young patients, with low survival rates, highlighting the need for comprehensive treatment protocols.
- Three cases treated between 2009 and 2014 utilized multimodal therapy with favorable outcomes for two patients, showing no disease progression, unlike most documented cases.
- Factors influencing a better prognosis include younger patient age, the tumor's location (supratentorial), and a reduction in complications from adjuvant treatments, supporting multidisciplinary approaches combining surgery, radiotherapy, and chemotherapy.
Article Abstract
Objective: Atypical teratoid rhabdoid tumors (AT/RT) of the central nervous system are rare, very aggressive embryological tumors, typically diagnosed in young patients and having a low survival rate after diagnosis. The aim of this study was to emphasize, based on the latest results in the literature, the need for protocols for multidisciplinary treatment in these patients.
Material And Methods: We report our series of 3 cases treated, diagnosed and followed up between 2009 and 2014. They were treated with multimodal therapy protocols (Rhabdoid SIOP-2007 and European Rhabdoid Registry EU-RHAB-2010). In addition, we carried out a literature review.
Results: Two of our 3 cases (supratentorial and spinal tumors) did not show any progression of the disease after long follow-up, in contrast with most of the cases available in the literature. The second patient had a shorter survival.
Conclusions: Patient age at the time of diagnosis, supratentorial location of the mass and fewer complications with adjuvant treatments seem to be factors yielding good prognosis for AT/RT tumors. In agreement with the latest international protocols, multidisciplinary treatment is the ideal treatment, consisting of radiotherapy and chemotherapy after complete tumor resection.
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| http://dx.doi.org/10.1016/j.neucir.2015.01.003 | DOI Listing |
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