PNR-16DIAGNOSIS, MANAGEMENT AND OUTCOME OF CHILDREN WITH CENTRAL NERVOUS SYSTEM (CNS) PRIMITIVE NEUROECTODERMAL TUMORS (PNET) IN SPAIN: A STUDY FROM THE SPANISH NATIONAL PEDIATRIC ONCOLOGY &amp; HEMATOLOGY SOCIETY (SEHOP)
Authors: Teresa de Rojas, Francisco José Bautista, Miguel Flores, Victoria Fioravantti, Lucía Igual, Raquel Rubio, Eduardo Bardón, Lucía Navarro, Laura Murillo, Raquel Hladun, Adela Cañete, Miguel García, Carmen Garrido, Ana Fernández-Teijeiro, Eduardo Quiroga, Carlota Calvo, Anna Llort, Luis Madero, Ofelia Cruz, Lucas Moreno
Journal: Neuro-Oncology (2016)

PNR-16. DIAGNOSIS, MANAGEMENT AND OUTCOME OF CHILDREN WITH CENTRAL NERVOUS SYSTEM (CNS) PRIMITIVE NEUROECTODERMAL TUMORS (PNET) IN SPAIN: A STUDY FROM THE SPANISH NATIONAL PEDIATRIC ONCOLOGY & HEMATOLOGY SOCIETY (SEHOP) Teresa de Rojas1, Francisco Jose Bautista1, Miguel Flores2, Victoria Fioravantti1, Lucia Igual3, Raquel Rubio4, Eduardo Bardon5, Lucia Navarro6, Laura Murillo7, Raquel Hladun8, Adela Canete3, Miguel Garcia4, Carmen Garrido5, Ana Fernandez-Teijeiro6, Eduardo Quiroga6, Carlota Calvo7, Anna Llort8, Luis Madero1, Ofelia Cruz2, and Lucas Moreno1; Hospital Infantil Universitario Nino Jesus, Madrid, Spain; Hospital Sant Joan deDeu,Barcelona, Spain; HospitalUniversitarioy Politecnico La Fe, Valencia, Spain; Hospital Universitario Cruces, Bilbao, Spain; Hospital General Universitario Gregorio Maranon, Madrid, Spain; Hospital Universitario Virgen del Rocio, Sevilla, Spain; Hospital Universitario Miguel Servet, Zaragoza, Spain; Hospital Universitario Vall d’Hebron, Barcelona, Spain BACKGROUND: CNS-PNET are aggressive embryonal tumors with poor outcome that have been historically treated in the context of medulloblastoma clinical trials. The purpose of this study is to present a tumor-specific, realworld data cohort of CNS-PNET. METHODS: Patients 0-21 years with CNS-PNET treated in 8 large institutions were included. Baseline characteristics, received treatment and outcomes [relapse-free and overall survival (RFS and OS respectively)] were analysed. RESULTS: 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 (79%). Median duration of the main symptom was 2 weeks (range 0-12). At diagnosis, 28% presented with metastatic disease. Primary tumor location was hemispheres (67%), pineal (12%), infratentorial (16.5%) and spinal cord (4.5%). Frontline treatment: 44% had gross total resection, 42% craniospinal radiotherapy, 86% chemotherapy with over 10 different strategies, and 33% autologous stem cell transplantation (aHSCT). Median follow-up for survivors was 3.5 years (range 1.7-9.3). 5-year RFS was 27.9% (95%CI 13-43%) and OS 30.7% (95%CI 16-46%). 73% of survivors had severe long-term sequelae. Age, M stage, extent of resection, radiotherapy and aHSCT were prognostic of OS in univariate analyisis (p , 0.05). CONCLUSIONS: Real-world data continues to show a dismal outcome for CNS-PNET despite advances in the field. In the current era, CNS-PNET is a distinct biological and genomic entity and new therapeutic strategies should be developed in multinational clinical trials. Neuro-Oncology 18:iii7–iii15, 2016. doi:10.1093/neuonc/now067.13 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.