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Spinal cord atypical teratoid/rhabdoid tumors in children: Clinical, genetic, and outcome characteristics in a representative European cohort
M. Benesch, K. Nemes, P. Neumayer, M. Hasselblatt, B. Timmermann, B. Bison, G. Ebetsberger-Dachs, F. Bourdeaut, C. Dufour, V. Biassoni, A. Morales La Madrid, N. Entz-Werle, V. Laithier, F. Quehenberger, S. Weis, D. Sumerauer, R. Siebert, S. Bens,...
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články, multicentrická studie, práce podpořená grantem
PubMed
31571386
DOI
10.1002/pbc.28022
Knihovny.cz E-zdroje
- MeSH
- dítě MeSH
- DNA-helikasy genetika MeSH
- gen SMARCB1 genetika MeSH
- jaderné proteiny genetika MeSH
- kojenec MeSH
- kombinovaná terapie MeSH
- lidé MeSH
- míra přežití MeSH
- nádorové biomarkery genetika MeSH
- nádory míchy genetika mortalita patologie terapie MeSH
- následné studie MeSH
- předškolní dítě MeSH
- prognóza MeSH
- retrospektivní studie MeSH
- rhabdoidní nádor genetika mortalita patologie terapie MeSH
- teratom genetika mortalita patologie terapie MeSH
- transkripční faktory genetika MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
BACKGROUND: Case reports have portrayed spinal cord atypical teratoid/rhabdoid tumor (spATRT) as an aggressive form of ATRT. We conducted a retrospective European survey to collect data on clinical characteristics, molecular biology, treatment, and outcome of children with intramedullary spATRT. METHODS: Scrutinizing a French national series and the European Rhabdoid Registry database, we identified 13 patients (median age 32 months; metastatic disease at diagnosis, n = 6). Systemic postoperative chemotherapy was administered to all patients; three received intrathecal therapy and six were irradiated (craniospinal, n = 3; local, n = 3). RESULTS: Median observation time was 8 (range, 1-93) months. Progression-free and overall survival rates at 1 and (2 years) were 35.2% ± 13.9% (26.4% ± 12.9%) and 38.5% ± 13.5% (23.1% ± 11.7%). Four patients (ATRT-SHH, n = 2; ATRT-MYC, n = 1; DNA methylation subgroup not available, n = 1) achieved complete remission (CR); two of them are alive in CR 69 and 72 months from diagnosis. One patient relapsed after CR and is alive with progressive disease (PD) and one died of the disease. Three patients (ATRT-MYC, n = 2; subgroup not available, n = 1) died after 7 to 22 months due to PD after having achieved a partial remission (n = 1) or stabilization (n = 2). Five patients (ATRT-MYC, n = 2; subgroup not available, n = 3) developed early PD and died. One patient (ATRT-MYC) died of intracerebral hemorrhage prior to response evaluation. CONCLUSIONS: Long-term survival is achievable in selected patients with spATRT using aggressive multimodality treatment. Larger case series and detailed molecular analyses are needed to understand differences between spATRT and their inracranial counterparts and the group of extradural malignant rhabdoid tumors.
Department of Pediatric Oncology University Hospital Besançon France
Hopp Children's Cancer Center Heidelberg Germany
Institute for Medical Statistics Medical University of Graz Graz Austria
Institute of Human Genetics Ulm University and Ulm University Medical Center Ulm Germany
Institute of Neuropathology University Hospital Münster Münster Germany
Laboratory of Genetics Pathology Unit S Anna General Hospital Como Italy
Oncology Department at Hospital Sant Joan de Deu Barcelona Children's Hospital Barcelona Spain
Pediatric Oncology Unit University Hospital Strasbourg France
PSL Research University Institut Curie Pediatric Care and Research Center Paris France
S C Pediatria Fondazione IRCCS Istituto Nazionale Tumori Milan Italy
University Hospital of Nancy Pediatric Hematology and Oncology Nancy France
Citace poskytuje Crossref.org
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