Ontology type: schema:ScholarlyArticle
2018-03
AUTHORSTeresa de Rojas, Francisco Bautista, Miguel Flores, Lucía Igual, Raquel Rubio, Eduardo Bardón, Lucía Navarro, Laura Murillo, Raquel Hladun, Adela Cañete, Miguel Garcia-Ariza, Carmen Garrido, Ana Fernández-Teijeiro, Eduardo Quiroga, Carlota Calvo, Anna Llort, Inmaculada de Prada, Luis Madero, Ofelia Cruz, Lucas Moreno
ABSTRACTNon-medulloblastoma CNS embryonal tumors (former PNET/Pineoblastomas) are aggressive malignancies with poor outcome that have been historically treated with medulloblastoma protocols. The purpose of this study is to present a tumor-specific, real-world data cohort of patients with CNS-PNET/PB to analyze quality indicators that can be implemented to improve the outcome of these patients. Patients 0-21 years with CNS-PNET treated in eight large institutions were included. Baseline characteristics, treatment and outcome [progression-free and overall survival (PFS and OS respectively)] were analyzed. From 2005 to 2014, 43 patients fulfilled entry criteria. Median age at diagnosis was 3.6 years (range 0.0-14.7). Histology was pineoblastoma (9%), ependymoblastoma (5%), ETANTR (7%) and PNET (77%). Median duration of the main symptom was 2 weeks (range 0-12). At diagnosis, 28% presented with metastatic disease. Seventeen different protocols were used on frontline treatment; 44% had gross total resection, 42% craniospinal radiotherapy, 86% chemotherapy, and 33% autologous hematopoietic stem cell transplantation (aHSCT). Median follow-up for survivors was 3.5 years (range 1.7-9.3). 3-year PFS was 31.9% (95% CI 17-47%) and OS 35.1% (95% CI 20-50%). Age, extent of resection and radiotherapy were prognostic of PFS and OS in univariate analysis (p < 0.05). Our series shows a dismal outcome for CNS-PNET, especially when compared to patients included in clinical trials. Establishing a common national strategy, implementing referral circuits and collaboration networks, and incorporating new molecular knowledge into routine clinical practice are accessible measures that can improve the outcome of these patients. More... »
PAGES205-213
http://scigraph.springernature.com/pub.10.1007/s11060-017-2713-4
DOIhttp://dx.doi.org/10.1007/s11060-017-2713-4
DIMENSIONShttps://app.dimensions.ai/details/publication/pub.1099689878
PUBMEDhttps://www.ncbi.nlm.nih.gov/pubmed/29248974
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