Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Benesch, M; Nemes, K; Neumayer, P; Hasselblatt, M; Timmermann, B; Bison, B; Ebetsberger-Dachs, G; Bourdeaut, F; Dufour, C; Biassoni, V; Morales La Madrid, A; Entz-Werle, N; Laithier, V; Quehenberger, F; Weis, S; Sumerauer, D; Siebert, R; Bens, S; Schneppenheim, R; Kool, M; Modena, P; Fouyssac, F; C Frühwald, M.
Spinal cord atypical teratoid/rhabdoid tumors in children: Clinical, genetic, and outcome characteristics in a representative European cohort.
Pediatr Blood Cancer. 2020; 67(1):e28022-e28022
Doi: 10.1002/pbc.28022
Web of Science
PubMed
FullText
FullText_MUG
- Führende Autor*innen der Med Uni Graz
- Benesch Martin
- Co-Autor*innen der Med Uni Graz
- Quehenberger Franz
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- 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. 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). 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. 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. © 2019 Wiley Periodicals, Inc.
- Find related publications in this database (using NLM MeSH Indexing)
- Biomarkers, Tumor - genetics
- Child -
- Child, Preschool -
- Combined Modality Therapy -
- DNA Helicases - genetics
- Female -
- Follow-Up Studies -
- Humans -
- Infant -
- Male -
- Nuclear Proteins - genetics
- Prognosis -
- Retrospective Studies -
- Rhabdoid Tumor - genetics
- Rhabdoid Tumor - mortality
- Rhabdoid Tumor - pathology
- Rhabdoid Tumor - therapy
- SMARCB1 Protein - genetics
- Spinal Cord Neoplasms - genetics
- Spinal Cord Neoplasms - mortality
- Spinal Cord Neoplasms - pathology
- Spinal Cord Neoplasms - therapy
- Survival Rate -
- Teratoma - genetics
- Teratoma - mortality
- Teratoma - pathology
- Teratoma - therapy
- Transcription Factors - genetics
- Find related publications in this database (Keywords)
- brain tumors
- neuro-oncology
- pediatric oncology
- teratoid
- rhabdoid tumors