Myxopapillary ependymoma in children: A study of 11 cases and a comparison with the adult experience
Background Myxopapillary ependymomas (MEPN) are uncommon tumors of the central nervous system, mainly occurring within the conus medullaris–filum terminale. MEPNs can also present in the skin and underlying soft tissues of the sacrococcygeal region. The incidence of extramedullary MEPNs in children...
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| Veröffentlicht in: | Pediatric blood & cancer 2014-11, Vol.61 (11), p.1969-1971 |
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| container_end_page | 1971 |
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| container_issue | 11 |
| container_start_page | 1969 |
| container_title | Pediatric blood & cancer |
| container_volume | 61 |
| creator | Cimino, Patrick J. Agarwal, Ashima Dehner, Louis P. |
| description | Background
Myxopapillary ependymomas (MEPN) are uncommon tumors of the central nervous system, mainly occurring within the conus medullaris–filum terminale. MEPNs can also present in the skin and underlying soft tissues of the sacrococcygeal region. The incidence of extramedullary MEPNs in children is unclear.
Procedure
We retrospectively reviewed our institutional files for MEPN cases between the years 1990–2012. A total of 11 pediatric and 38 adult cases were identified and compared to determine differences between these two distinct age groups.
Results
There were 40 (82%) tumors arising in the spine and nine (18%) in extramedullary sites. Pathologic examination revealed that extramedullary and spinal MEPNs were indistinguishable morphologically and immunophenotypically. Among the 11 children with MEPNs, seven had tumors presenting in extramedullary sites whereas only two adults (5%) had extramedullary tumors; this difference was highly significant (P |
| doi_str_mv | 10.1002/pbc.25125 |
| format | Article |
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Myxopapillary ependymomas (MEPN) are uncommon tumors of the central nervous system, mainly occurring within the conus medullaris–filum terminale. MEPNs can also present in the skin and underlying soft tissues of the sacrococcygeal region. The incidence of extramedullary MEPNs in children is unclear.
Procedure
We retrospectively reviewed our institutional files for MEPN cases between the years 1990–2012. A total of 11 pediatric and 38 adult cases were identified and compared to determine differences between these two distinct age groups.
Results
There were 40 (82%) tumors arising in the spine and nine (18%) in extramedullary sites. Pathologic examination revealed that extramedullary and spinal MEPNs were indistinguishable morphologically and immunophenotypically. Among the 11 children with MEPNs, seven had tumors presenting in extramedullary sites whereas only two adults (5%) had extramedullary tumors; this difference was highly significant (P < 0.0001). The lumbosacral portion of the spinal cord was the primary site for the remaining 40 MEPNs, four (10%) of which presented in children. One extramedullary MEPN (9%) recurred in a 45‐year‐old woman, and five (11%) of 40 spinal tumors recurred and/or metastasized; two of four children with spinal MEPNs developed recurrences and a manifestation to the aggressive nature of the latter tumor in the spinal cord of children.
Conclusions
Based upon our experience, MEPN in children is more likely to present in the extramedullary soft tissues of the sacrococcygeal region where its behavior is more indolent than those tumors arising in the spinal cord in children. Pediatr Blood Cancer 2014;61:1969–1971. © 2014 Wiley Periodicals, Inc.</description><identifier>ISSN: 1545-5009</identifier><identifier>EISSN: 1545-5017</identifier><identifier>DOI: 10.1002/pbc.25125</identifier><identifier>PMID: 25066546</identifier><language>eng</language><publisher>United States: Blackwell Publishing Ltd</publisher><subject>Adolescent ; Adult ; Aged ; central nervous system ; Child ; Child, Preschool ; ependymoma ; Ependymoma - pathology ; Female ; Hematology ; Humans ; Infant ; Male ; Middle Aged ; myxopapillary ependymoma ; Oncology ; Pediatrics ; Retrospective Studies ; sacrococcygeal region ; Soft Tissue Neoplasms - pathology ; spinal cord ; Spinal Cord Neoplasms - pathology</subject><ispartof>Pediatric blood & cancer, 2014-11, Vol.61 (11), p.1969-1971</ispartof><rights>2014 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3915-26c59a63daea905cb5b1235662e9f578a2094f4d9ac7735dac596a5b7aa6fe7a3</citedby><cites>FETCH-LOGICAL-c3915-26c59a63daea905cb5b1235662e9f578a2094f4d9ac7735dac596a5b7aa6fe7a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fpbc.25125$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fpbc.25125$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27903,27904,45553,45554</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25066546$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Cimino, Patrick J.</creatorcontrib><creatorcontrib>Agarwal, Ashima</creatorcontrib><creatorcontrib>Dehner, Louis P.</creatorcontrib><title>Myxopapillary ependymoma in children: A study of 11 cases and a comparison with the adult experience</title><title>Pediatric blood & cancer</title><addtitle>Pediatr Blood Cancer</addtitle><description>Background
Myxopapillary ependymomas (MEPN) are uncommon tumors of the central nervous system, mainly occurring within the conus medullaris–filum terminale. MEPNs can also present in the skin and underlying soft tissues of the sacrococcygeal region. The incidence of extramedullary MEPNs in children is unclear.
Procedure
We retrospectively reviewed our institutional files for MEPN cases between the years 1990–2012. A total of 11 pediatric and 38 adult cases were identified and compared to determine differences between these two distinct age groups.
Results
There were 40 (82%) tumors arising in the spine and nine (18%) in extramedullary sites. Pathologic examination revealed that extramedullary and spinal MEPNs were indistinguishable morphologically and immunophenotypically. Among the 11 children with MEPNs, seven had tumors presenting in extramedullary sites whereas only two adults (5%) had extramedullary tumors; this difference was highly significant (P < 0.0001). The lumbosacral portion of the spinal cord was the primary site for the remaining 40 MEPNs, four (10%) of which presented in children. One extramedullary MEPN (9%) recurred in a 45‐year‐old woman, and five (11%) of 40 spinal tumors recurred and/or metastasized; two of four children with spinal MEPNs developed recurrences and a manifestation to the aggressive nature of the latter tumor in the spinal cord of children.
Conclusions
Based upon our experience, MEPN in children is more likely to present in the extramedullary soft tissues of the sacrococcygeal region where its behavior is more indolent than those tumors arising in the spinal cord in children. Pediatr Blood Cancer 2014;61:1969–1971. © 2014 Wiley Periodicals, Inc.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>central nervous system</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>ependymoma</subject><subject>Ependymoma - pathology</subject><subject>Female</subject><subject>Hematology</subject><subject>Humans</subject><subject>Infant</subject><subject>Male</subject><subject>Middle Aged</subject><subject>myxopapillary ependymoma</subject><subject>Oncology</subject><subject>Pediatrics</subject><subject>Retrospective Studies</subject><subject>sacrococcygeal region</subject><subject>Soft Tissue Neoplasms - pathology</subject><subject>spinal cord</subject><subject>Spinal Cord Neoplasms - pathology</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10E1v1DAQBuAIgegXB_4AssSFHtLaccZJuJUVFKQWWgriaE3sidYlX9iJuvn3uGy7h0qcPIdnXo3fJHkt-IngPDsda3OSgcjgWbIvIIcUuCie72Ze7SUHIdxGqjiUL5O9DLhSkKv9xF4um2HE0bUt-oXRSL1duqFD5npm1q61nvr37IyFabYLGxomBDMYKDDsLUNmhm5E78LQszs3rdm0JoZ2bidGm5G8o97QUfKiwTbQq4f3MPn56eOP1ef04tv5l9XZRWpkJSDNlIEKlbRIWHEwNdQik6BURlUDRYkZr_ImtxWaopBgMXKFUBeIqqEC5WHybps7-uHPTGHSnQuG4td6GuagBahcggSoIn37hN4Os-_jdfdKlmWVizKq460yfgjBU6NH77pYlBZc31evY_X6X_XRvnlInOuO7E4-dh3B6RbcuZaW_yfpqw-rx8h0u-HCRJvdBvrfWhWyAP3r67n-nl1zKW5u9LX8C1n3m5s</recordid><startdate>201411</startdate><enddate>201411</enddate><creator>Cimino, Patrick J.</creator><creator>Agarwal, Ashima</creator><creator>Dehner, Louis P.</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</scope><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>7T5</scope><scope>7TK</scope><scope>7TO</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>K9.</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope></search><sort><creationdate>201411</creationdate><title>Myxopapillary ependymoma in children: A study of 11 cases and a comparison with the adult experience</title><author>Cimino, Patrick J. ; Agarwal, Ashima ; Dehner, Louis P.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3915-26c59a63daea905cb5b1235662e9f578a2094f4d9ac7735dac596a5b7aa6fe7a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>central nervous system</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>ependymoma</topic><topic>Ependymoma - pathology</topic><topic>Female</topic><topic>Hematology</topic><topic>Humans</topic><topic>Infant</topic><topic>Male</topic><topic>Middle Aged</topic><topic>myxopapillary ependymoma</topic><topic>Oncology</topic><topic>Pediatrics</topic><topic>Retrospective Studies</topic><topic>sacrococcygeal region</topic><topic>Soft Tissue Neoplasms - pathology</topic><topic>spinal cord</topic><topic>Spinal Cord Neoplasms - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Cimino, Patrick J.</creatorcontrib><creatorcontrib>Agarwal, Ashima</creatorcontrib><creatorcontrib>Dehner, Louis P.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric blood & cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Cimino, Patrick J.</au><au>Agarwal, Ashima</au><au>Dehner, Louis P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Myxopapillary ependymoma in children: A study of 11 cases and a comparison with the adult experience</atitle><jtitle>Pediatric blood & cancer</jtitle><addtitle>Pediatr Blood Cancer</addtitle><date>2014-11</date><risdate>2014</risdate><volume>61</volume><issue>11</issue><spage>1969</spage><epage>1971</epage><pages>1969-1971</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><abstract>Background
Myxopapillary ependymomas (MEPN) are uncommon tumors of the central nervous system, mainly occurring within the conus medullaris–filum terminale. MEPNs can also present in the skin and underlying soft tissues of the sacrococcygeal region. The incidence of extramedullary MEPNs in children is unclear.
Procedure
We retrospectively reviewed our institutional files for MEPN cases between the years 1990–2012. A total of 11 pediatric and 38 adult cases were identified and compared to determine differences between these two distinct age groups.
Results
There were 40 (82%) tumors arising in the spine and nine (18%) in extramedullary sites. Pathologic examination revealed that extramedullary and spinal MEPNs were indistinguishable morphologically and immunophenotypically. Among the 11 children with MEPNs, seven had tumors presenting in extramedullary sites whereas only two adults (5%) had extramedullary tumors; this difference was highly significant (P < 0.0001). The lumbosacral portion of the spinal cord was the primary site for the remaining 40 MEPNs, four (10%) of which presented in children. One extramedullary MEPN (9%) recurred in a 45‐year‐old woman, and five (11%) of 40 spinal tumors recurred and/or metastasized; two of four children with spinal MEPNs developed recurrences and a manifestation to the aggressive nature of the latter tumor in the spinal cord of children.
Conclusions
Based upon our experience, MEPN in children is more likely to present in the extramedullary soft tissues of the sacrococcygeal region where its behavior is more indolent than those tumors arising in the spinal cord in children. Pediatr Blood Cancer 2014;61:1969–1971. © 2014 Wiley Periodicals, Inc.</abstract><cop>United States</cop><pub>Blackwell Publishing Ltd</pub><pmid>25066546</pmid><doi>10.1002/pbc.25125</doi><tpages>3</tpages></addata></record> |
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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Adolescent Adult Aged central nervous system Child Child, Preschool ependymoma Ependymoma - pathology Female Hematology Humans Infant Male Middle Aged myxopapillary ependymoma Oncology Pediatrics Retrospective Studies sacrococcygeal region Soft Tissue Neoplasms - pathology spinal cord Spinal Cord Neoplasms - pathology |
| title | Myxopapillary ependymoma in children: A study of 11 cases and a comparison with the adult experience |
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