Portal de investigación
Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care
Autores de IIS La Fe
Participantes ajenos a IIS La Fe
- de Rojas, T
- Bautista, F
- Flores, M
- Rubio, R
- Bardon, E
- Navarro, L
- Murillo, L
- Hladun, R
- Garcia-Ariza, M
- Garrido, C
- Fernandez-Teijeiro, A
- Quiroga, E
- Calvo, C
- Llort, A
- de Prada, I
- Madero, L
- Cruz, O
- Moreno, L
Grupos
Abstract
Non-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.
Datos de la publicación
- ISSN/ISSNe:
- 0167-594X, 1573-7373
- Tipo:
- Article
- Páginas:
- 205-213
- PubMed:
- 29248974
- Factor de Impacto:
- 1,194 SCImago ℠
- Cuartil:
- Q1 SCImago ℠
JOURNAL OF NEURO-ONCOLOGY SPRINGER
Citas Recibidas en Web of Science: 7
Documentos
- No hay documentos
Filiaciones
Keywords
- Central nervous system tumors; Embryonal tumors; PNET; Pineoblastoma; Outcome; Children
Campos de Estudio
Cita
de Rojas T,Bautista F,Flores M,IGUAL L,Rubio R,Bardon E,Navarro L,Murillo L,Hladun R,CAÑETE A,Garcia M,Garrido C,Fernandez A,Quiroga E,Calvo C,Llort A,de Prada I,Madero L,Cruz O,Moreno L. Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care. J. Neuro-Oncol. 2018. 137. (1):p. 205-213. IF:3,129. (2).