Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care
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 ana...
Gespeichert in:
| Veröffentlicht in: | Journal of neuro-oncology 2018-03, Vol.137 (1), p.205-213 |
|---|---|
| Hauptverfasser: | , , , , , , , , , , , , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 213 |
|---|---|
| container_issue | 1 |
| container_start_page | 205 |
| container_title | Journal of neuro-oncology |
| container_volume | 137 |
| creator | de Rojas, Teresa Bautista, Francisco Flores, Miguel Igual, Lucía Rubio, Raquel Bardón, Eduardo Navarro, Lucía Murillo, Laura Hladun, Raquel Cañete, Adela Garcia-Ariza, Miguel Garrido, Carmen Fernández-Teijeiro, Ana Quiroga, Eduardo Calvo, Carlota Llort, Anna de Prada, Inmaculada Madero, Luis Cruz, Ofelia Moreno, Lucas |
| description | 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 |
| doi_str_mv | 10.1007/s11060-017-2713-4 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1978320752</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1977616322</sourcerecordid><originalsourceid>FETCH-LOGICAL-c372t-fb4f9ae6f24e68a5d0a55d61ab142ffe447451ab2fd76d87f101cdfcf6d1af413</originalsourceid><addsrcrecordid>eNp1kc2OFCEUhYnROD2jD-DGkLhxg3KBgi53pqOjyaiL0cQdoQqYqQk_LVRp5j18YOmu0RgTVwTud8-5l4PQE6AvgFL1sgJQSQkFRZgCTsQ9tIFOcaK44vfRhoJUpOvF1xN0WusNpVQoDg_RCeuZ2PZKbNDPDyaZKxddmrFJFudlHnN0OHs8Xk_BFpeO78bm4OrYsIp_TPM1TjmR6OwSQh6CqXOOBu8-XmIXh3Kbkwl4XmIuFU8JX-7NlF7hknPEPhc8xX3J31fTVq5zczDF1qOrKe4ReuBNqO7x3XmGvrx983n3jlx8On-_e31BRq7YTPwgfG-c9Ew4uTWdpabrrAQzgGDeOyGU6NqNeauk3SoPFEbrRy8tGC-An6Hnq24b59vi6qzj1HYMwSSXl6qhV1vOqOpYQ5_9g97kpbQ1j5SSIDk7ULBSY8m1Fuf1vkzRlFsNVB8i02tkukWmD5Fp0Xqe3ikvQ_vQPx2_M2oAW4HaSunKlb-s_6v6C02WpEs</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1977616322</pqid></control><display><type>article</type><title>Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care</title><source>MEDLINE</source><source>Springer Nature - Complete Springer Journals</source><creator>de Rojas, Teresa ; Bautista, Francisco ; Flores, Miguel ; Igual, Lucía ; Rubio, Raquel ; Bardón, Eduardo ; Navarro, Lucía ; Murillo, Laura ; Hladun, Raquel ; Cañete, Adela ; Garcia-Ariza, Miguel ; Garrido, Carmen ; Fernández-Teijeiro, Ana ; Quiroga, Eduardo ; Calvo, Carlota ; Llort, Anna ; de Prada, Inmaculada ; Madero, Luis ; Cruz, Ofelia ; Moreno, Lucas</creator><creatorcontrib>de Rojas, Teresa ; Bautista, Francisco ; Flores, Miguel ; Igual, Lucía ; Rubio, Raquel ; Bardón, Eduardo ; Navarro, Lucía ; Murillo, Laura ; Hladun, Raquel ; Cañete, Adela ; Garcia-Ariza, Miguel ; Garrido, Carmen ; Fernández-Teijeiro, Ana ; Quiroga, Eduardo ; Calvo, Carlota ; Llort, Anna ; de Prada, Inmaculada ; Madero, Luis ; Cruz, Ofelia ; Moreno, Lucas</creatorcontrib><description>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.</description><identifier>ISSN: 0167-594X</identifier><identifier>EISSN: 1573-7373</identifier><identifier>DOI: 10.1007/s11060-017-2713-4</identifier><identifier>PMID: 29248974</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Adolescent ; Adolescents ; Autografts ; Brain cancer ; Brain Neoplasms - diagnosis ; Brain Neoplasms - therapy ; Central nervous system ; Chemotherapy ; Child ; Child, Preschool ; Children ; Clinical Study ; Clinical trials ; Cohort Studies ; Diagnosis ; Female ; Hematopoietic stem cells ; Humans ; Male ; Medicine ; Medicine & Public Health ; Medulloblastoma ; Metastases ; Neoplasia ; Neurology ; Oncology ; Outcome and Process Assessment (Health Care) ; Pineal gland ; Pinealoma - diagnosis ; Pinealoma - therapy ; Radiation therapy ; Spain ; Standard of Care ; Stem cell transplantation ; Survival Analysis ; Transplantation ; Treatment Outcome ; Tumors</subject><ispartof>Journal of neuro-oncology, 2018-03, Vol.137 (1), p.205-213</ispartof><rights>Springer Science+Business Media, LLC, part of Springer Nature 2017</rights><rights>Journal of Neuro-Oncology is a copyright of Springer, (2017). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c372t-fb4f9ae6f24e68a5d0a55d61ab142ffe447451ab2fd76d87f101cdfcf6d1af413</citedby><cites>FETCH-LOGICAL-c372t-fb4f9ae6f24e68a5d0a55d61ab142ffe447451ab2fd76d87f101cdfcf6d1af413</cites><orcidid>0000-0002-0708-1670</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s11060-017-2713-4$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s11060-017-2713-4$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29248974$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>de Rojas, Teresa</creatorcontrib><creatorcontrib>Bautista, Francisco</creatorcontrib><creatorcontrib>Flores, Miguel</creatorcontrib><creatorcontrib>Igual, Lucía</creatorcontrib><creatorcontrib>Rubio, Raquel</creatorcontrib><creatorcontrib>Bardón, Eduardo</creatorcontrib><creatorcontrib>Navarro, Lucía</creatorcontrib><creatorcontrib>Murillo, Laura</creatorcontrib><creatorcontrib>Hladun, Raquel</creatorcontrib><creatorcontrib>Cañete, Adela</creatorcontrib><creatorcontrib>Garcia-Ariza, Miguel</creatorcontrib><creatorcontrib>Garrido, Carmen</creatorcontrib><creatorcontrib>Fernández-Teijeiro, Ana</creatorcontrib><creatorcontrib>Quiroga, Eduardo</creatorcontrib><creatorcontrib>Calvo, Carlota</creatorcontrib><creatorcontrib>Llort, Anna</creatorcontrib><creatorcontrib>de Prada, Inmaculada</creatorcontrib><creatorcontrib>Madero, Luis</creatorcontrib><creatorcontrib>Cruz, Ofelia</creatorcontrib><creatorcontrib>Moreno, Lucas</creatorcontrib><title>Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care</title><title>Journal of neuro-oncology</title><addtitle>J Neurooncol</addtitle><addtitle>J Neurooncol</addtitle><description>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.</description><subject>Adolescent</subject><subject>Adolescents</subject><subject>Autografts</subject><subject>Brain cancer</subject><subject>Brain Neoplasms - diagnosis</subject><subject>Brain Neoplasms - therapy</subject><subject>Central nervous system</subject><subject>Chemotherapy</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Clinical Study</subject><subject>Clinical trials</subject><subject>Cohort Studies</subject><subject>Diagnosis</subject><subject>Female</subject><subject>Hematopoietic stem cells</subject><subject>Humans</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Medulloblastoma</subject><subject>Metastases</subject><subject>Neoplasia</subject><subject>Neurology</subject><subject>Oncology</subject><subject>Outcome and Process Assessment (Health Care)</subject><subject>Pineal gland</subject><subject>Pinealoma - diagnosis</subject><subject>Pinealoma - therapy</subject><subject>Radiation therapy</subject><subject>Spain</subject><subject>Standard of Care</subject><subject>Stem cell transplantation</subject><subject>Survival Analysis</subject><subject>Transplantation</subject><subject>Treatment Outcome</subject><subject>Tumors</subject><issn>0167-594X</issn><issn>1573-7373</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp1kc2OFCEUhYnROD2jD-DGkLhxg3KBgi53pqOjyaiL0cQdoQqYqQk_LVRp5j18YOmu0RgTVwTud8-5l4PQE6AvgFL1sgJQSQkFRZgCTsQ9tIFOcaK44vfRhoJUpOvF1xN0WusNpVQoDg_RCeuZ2PZKbNDPDyaZKxddmrFJFudlHnN0OHs8Xk_BFpeO78bm4OrYsIp_TPM1TjmR6OwSQh6CqXOOBu8-XmIXh3Kbkwl4XmIuFU8JX-7NlF7hknPEPhc8xX3J31fTVq5zczDF1qOrKe4ReuBNqO7x3XmGvrx983n3jlx8On-_e31BRq7YTPwgfG-c9Ew4uTWdpabrrAQzgGDeOyGU6NqNeauk3SoPFEbrRy8tGC-An6Hnq24b59vi6qzj1HYMwSSXl6qhV1vOqOpYQ5_9g97kpbQ1j5SSIDk7ULBSY8m1Fuf1vkzRlFsNVB8i02tkukWmD5Fp0Xqe3ikvQ_vQPx2_M2oAW4HaSunKlb-s_6v6C02WpEs</recordid><startdate>20180301</startdate><enddate>20180301</enddate><creator>de Rojas, Teresa</creator><creator>Bautista, Francisco</creator><creator>Flores, Miguel</creator><creator>Igual, Lucía</creator><creator>Rubio, Raquel</creator><creator>Bardón, Eduardo</creator><creator>Navarro, Lucía</creator><creator>Murillo, Laura</creator><creator>Hladun, Raquel</creator><creator>Cañete, Adela</creator><creator>Garcia-Ariza, Miguel</creator><creator>Garrido, Carmen</creator><creator>Fernández-Teijeiro, Ana</creator><creator>Quiroga, Eduardo</creator><creator>Calvo, Carlota</creator><creator>Llort, Anna</creator><creator>de Prada, Inmaculada</creator><creator>Madero, Luis</creator><creator>Cruz, Ofelia</creator><creator>Moreno, Lucas</creator><general>Springer US</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-0708-1670</orcidid></search><sort><creationdate>20180301</creationdate><title>Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care</title><author>de Rojas, Teresa ; Bautista, Francisco ; Flores, Miguel ; Igual, Lucía ; Rubio, Raquel ; Bardón, Eduardo ; Navarro, Lucía ; Murillo, Laura ; Hladun, Raquel ; Cañete, Adela ; Garcia-Ariza, Miguel ; Garrido, Carmen ; Fernández-Teijeiro, Ana ; Quiroga, Eduardo ; Calvo, Carlota ; Llort, Anna ; de Prada, Inmaculada ; Madero, Luis ; Cruz, Ofelia ; Moreno, Lucas</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c372t-fb4f9ae6f24e68a5d0a55d61ab142ffe447451ab2fd76d87f101cdfcf6d1af413</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Adolescent</topic><topic>Adolescents</topic><topic>Autografts</topic><topic>Brain cancer</topic><topic>Brain Neoplasms - diagnosis</topic><topic>Brain Neoplasms - therapy</topic><topic>Central nervous system</topic><topic>Chemotherapy</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Children</topic><topic>Clinical Study</topic><topic>Clinical trials</topic><topic>Cohort Studies</topic><topic>Diagnosis</topic><topic>Female</topic><topic>Hematopoietic stem cells</topic><topic>Humans</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Medulloblastoma</topic><topic>Metastases</topic><topic>Neoplasia</topic><topic>Neurology</topic><topic>Oncology</topic><topic>Outcome and Process Assessment (Health Care)</topic><topic>Pineal gland</topic><topic>Pinealoma - diagnosis</topic><topic>Pinealoma - therapy</topic><topic>Radiation therapy</topic><topic>Spain</topic><topic>Standard of Care</topic><topic>Stem cell transplantation</topic><topic>Survival Analysis</topic><topic>Transplantation</topic><topic>Treatment Outcome</topic><topic>Tumors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>de Rojas, Teresa</creatorcontrib><creatorcontrib>Bautista, Francisco</creatorcontrib><creatorcontrib>Flores, Miguel</creatorcontrib><creatorcontrib>Igual, Lucía</creatorcontrib><creatorcontrib>Rubio, Raquel</creatorcontrib><creatorcontrib>Bardón, Eduardo</creatorcontrib><creatorcontrib>Navarro, Lucía</creatorcontrib><creatorcontrib>Murillo, Laura</creatorcontrib><creatorcontrib>Hladun, Raquel</creatorcontrib><creatorcontrib>Cañete, Adela</creatorcontrib><creatorcontrib>Garcia-Ariza, Miguel</creatorcontrib><creatorcontrib>Garrido, Carmen</creatorcontrib><creatorcontrib>Fernández-Teijeiro, Ana</creatorcontrib><creatorcontrib>Quiroga, Eduardo</creatorcontrib><creatorcontrib>Calvo, Carlota</creatorcontrib><creatorcontrib>Llort, Anna</creatorcontrib><creatorcontrib>de Prada, Inmaculada</creatorcontrib><creatorcontrib>Madero, Luis</creatorcontrib><creatorcontrib>Cruz, Ofelia</creatorcontrib><creatorcontrib>Moreno, Lucas</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of neuro-oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>de Rojas, Teresa</au><au>Bautista, Francisco</au><au>Flores, Miguel</au><au>Igual, Lucía</au><au>Rubio, Raquel</au><au>Bardón, Eduardo</au><au>Navarro, Lucía</au><au>Murillo, Laura</au><au>Hladun, Raquel</au><au>Cañete, Adela</au><au>Garcia-Ariza, Miguel</au><au>Garrido, Carmen</au><au>Fernández-Teijeiro, Ana</au><au>Quiroga, Eduardo</au><au>Calvo, Carlota</au><au>Llort, Anna</au><au>de Prada, Inmaculada</au><au>Madero, Luis</au><au>Cruz, Ofelia</au><au>Moreno, Lucas</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care</atitle><jtitle>Journal of neuro-oncology</jtitle><stitle>J Neurooncol</stitle><addtitle>J Neurooncol</addtitle><date>2018-03-01</date><risdate>2018</risdate><volume>137</volume><issue>1</issue><spage>205</spage><epage>213</epage><pages>205-213</pages><issn>0167-594X</issn><eissn>1573-7373</eissn><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.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>29248974</pmid><doi>10.1007/s11060-017-2713-4</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-0708-1670</orcidid></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0167-594X |
| ispartof | Journal of neuro-oncology, 2018-03, Vol.137 (1), p.205-213 |
| issn | 0167-594X 1573-7373 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_1978320752 |
| source | MEDLINE; Springer Nature - Complete Springer Journals |
| subjects | Adolescent Adolescents Autografts Brain cancer Brain Neoplasms - diagnosis Brain Neoplasms - therapy Central nervous system Chemotherapy Child Child, Preschool Children Clinical Study Clinical trials Cohort Studies Diagnosis Female Hematopoietic stem cells Humans Male Medicine Medicine & Public Health Medulloblastoma Metastases Neoplasia Neurology Oncology Outcome and Process Assessment (Health Care) Pineal gland Pinealoma - diagnosis Pinealoma - therapy Radiation therapy Spain Standard of Care Stem cell transplantation Survival Analysis Transplantation Treatment Outcome Tumors |
| title | Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-12T16%3A42%3A26IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Management%20and%20outcome%20of%20children%20and%20adolescents%20with%20non-medulloblastoma%20CNS%20embryonal%20tumors%20in%20Spain:%20room%20for%20improvement%20in%20standards%20of%20care&rft.jtitle=Journal%20of%20neuro-oncology&rft.au=de%20Rojas,%20Teresa&rft.date=2018-03-01&rft.volume=137&rft.issue=1&rft.spage=205&rft.epage=213&rft.pages=205-213&rft.issn=0167-594X&rft.eissn=1573-7373&rft_id=info:doi/10.1007/s11060-017-2713-4&rft_dat=%3Cproquest_cross%3E1977616322%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1977616322&rft_id=info:pmid/29248974&rfr_iscdi=true |