Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)
BACKGROUND Surgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the...
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Veröffentlicht in: | Cancer 2006-05, Vol.106 (10), p.2275-2283 |
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creator | Furtwaengler, Rhoikos Reinhard, Harald Leuschner, Ivo Schenk, Jens P. Goebel, Ulrich Claviez, Alexander Kulozik, Andreas Zoubek, Andreas von Schweinitz, Dietrich Graf, Norbert |
description | BACKGROUND
Surgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93‐01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.
METHODS
In total, 50 patients with MN were analyzed. Eleven patients were suspected antenatally of having a renal tumor. The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years.
RESULTS
Forty‐five patients underwent initial surgery. Five patients older than 6 months received preoperative chemotherapy. Twenty‐nine tumors were classic MN, and 21 tumors were cellular MN. Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 patients received no further treatment. For the entire group, event‐free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS. Three patients developed recurrent disease, and 2 of those patients died. Metastases to the brain, lung, and liver were observed in 1 patient.
CONCLUSIONS
Radical nephrectomy with accurate surgical‐pathologic staging is the standard of care for children with MN. Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy. Cancer 2006. © 2006 American Cancer Society.
The authors report on their analysis of prospectively collected data from 50 patients with a mesoblastic nephroma (MN). The findings indicated that patients who have the cellular subtype of MN in combination with Stage III disease and older age are at risk for recurrence and therefore may profit from adjuvant treatment. |
doi_str_mv | 10.1002/cncr.21836 |
format | Article |
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Surgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93‐01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.
METHODS
In total, 50 patients with MN were analyzed. Eleven patients were suspected antenatally of having a renal tumor. The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years.
RESULTS
Forty‐five patients underwent initial surgery. Five patients older than 6 months received preoperative chemotherapy. Twenty‐nine tumors were classic MN, and 21 tumors were cellular MN. Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 patients received no further treatment. For the entire group, event‐free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS. Three patients developed recurrent disease, and 2 of those patients died. Metastases to the brain, lung, and liver were observed in 1 patient.
CONCLUSIONS
Radical nephrectomy with accurate surgical‐pathologic staging is the standard of care for children with MN. Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy. Cancer 2006. © 2006 American Cancer Society.
The authors report on their analysis of prospectively collected data from 50 patients with a mesoblastic nephroma (MN). The findings indicated that patients who have the cellular subtype of MN in combination with Stage III disease and older age are at risk for recurrence and therefore may profit from adjuvant treatment.</description><identifier>ISSN: 0008-543X</identifier><identifier>EISSN: 1097-0142</identifier><identifier>DOI: 10.1002/cncr.21836</identifier><identifier>PMID: 16596620</identifier><identifier>CODEN: CANCAR</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>Age Factors ; Biological and medical sciences ; Biopsy, Needle ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; childhood renal tumor ; Cohort Studies ; Female ; Germany ; Gesellschaft fur Pädiatrische Onkologie und Hämatologie ; Humans ; Immunohistochemistry ; Infant ; Infant, Newborn ; International Society of Pediatric Oncology ; Kidney Neoplasms - diagnosis ; Kidney Neoplasms - mortality ; Kidney Neoplasms - therapy ; Kidneys ; Logistic Models ; Male ; Medical sciences ; mesoblastic nephroma ; multicenter study ; neonate ; Neoplasm Staging ; Nephrectomy - methods ; Nephrology. Urinary tract diseases ; Nephroma, Mesoblastic - diagnosis ; Nephroma, Mesoblastic - mortality ; Nephroma, Mesoblastic - therapy ; pediatric oncology ; Pediatrics ; prenatal diagnosis ; Prognosis ; Proportional Hazards Models ; Retrospective Studies ; Risk Assessment ; Societies, Medical ; Statistics, Nonparametric ; Survival Analysis ; Treatment Outcome ; Tumors ; Tumors of the urinary system</subject><ispartof>Cancer, 2006-05, Vol.106 (10), p.2275-2283</ispartof><rights>Copyright © 2006 American Cancer Society</rights><rights>2006 INIST-CNRS</rights><rights>Copyright 2006 American Cancer Society</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3936-31b8977366177c88c25453fed49ea049fccde82755553f5d6a9ccf1df96c38db3</citedby><cites>FETCH-LOGICAL-c3936-31b8977366177c88c25453fed49ea049fccde82755553f5d6a9ccf1df96c38db3</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%2Fcncr.21836$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fcncr.21836$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,1427,27901,27902,45550,45551,46384,46808</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=17729512$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16596620$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Furtwaengler, Rhoikos</creatorcontrib><creatorcontrib>Reinhard, Harald</creatorcontrib><creatorcontrib>Leuschner, Ivo</creatorcontrib><creatorcontrib>Schenk, Jens P.</creatorcontrib><creatorcontrib>Goebel, Ulrich</creatorcontrib><creatorcontrib>Claviez, Alexander</creatorcontrib><creatorcontrib>Kulozik, Andreas</creatorcontrib><creatorcontrib>Zoubek, Andreas</creatorcontrib><creatorcontrib>von Schweinitz, Dietrich</creatorcontrib><creatorcontrib>Graf, Norbert</creatorcontrib><creatorcontrib>Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH) Nephroblastoma Study Group</creatorcontrib><title>Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)</title><title>Cancer</title><addtitle>Cancer</addtitle><description>BACKGROUND
Surgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93‐01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.
METHODS
In total, 50 patients with MN were analyzed. Eleven patients were suspected antenatally of having a renal tumor. The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years.
RESULTS
Forty‐five patients underwent initial surgery. Five patients older than 6 months received preoperative chemotherapy. Twenty‐nine tumors were classic MN, and 21 tumors were cellular MN. Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 patients received no further treatment. For the entire group, event‐free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS. Three patients developed recurrent disease, and 2 of those patients died. Metastases to the brain, lung, and liver were observed in 1 patient.
CONCLUSIONS
Radical nephrectomy with accurate surgical‐pathologic staging is the standard of care for children with MN. Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy. Cancer 2006. © 2006 American Cancer Society.
The authors report on their analysis of prospectively collected data from 50 patients with a mesoblastic nephroma (MN). The findings indicated that patients who have the cellular subtype of MN in combination with Stage III disease and older age are at risk for recurrence and therefore may profit from adjuvant treatment.</description><subject>Age Factors</subject><subject>Biological and medical sciences</subject><subject>Biopsy, Needle</subject><subject>Chemotherapy, Adjuvant</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>childhood renal tumor</subject><subject>Cohort Studies</subject><subject>Female</subject><subject>Germany</subject><subject>Gesellschaft fur Pädiatrische Onkologie und Hämatologie</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>International Society of Pediatric Oncology</subject><subject>Kidney Neoplasms - diagnosis</subject><subject>Kidney Neoplasms - mortality</subject><subject>Kidney Neoplasms - therapy</subject><subject>Kidneys</subject><subject>Logistic Models</subject><subject>Male</subject><subject>Medical sciences</subject><subject>mesoblastic nephroma</subject><subject>multicenter study</subject><subject>neonate</subject><subject>Neoplasm Staging</subject><subject>Nephrectomy - methods</subject><subject>Nephrology. Urinary tract diseases</subject><subject>Nephroma, Mesoblastic - diagnosis</subject><subject>Nephroma, Mesoblastic - mortality</subject><subject>Nephroma, Mesoblastic - therapy</subject><subject>pediatric oncology</subject><subject>Pediatrics</subject><subject>prenatal diagnosis</subject><subject>Prognosis</subject><subject>Proportional Hazards Models</subject><subject>Retrospective Studies</subject><subject>Risk Assessment</subject><subject>Societies, Medical</subject><subject>Statistics, Nonparametric</subject><subject>Survival Analysis</subject><subject>Treatment Outcome</subject><subject>Tumors</subject><subject>Tumors of the urinary system</subject><issn>0008-543X</issn><issn>1097-0142</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE1KBDEQhYMoOv5sPIBko6jQmp_upLOUQWcEdUQU3DWZdMVp7Z8x6UbceQiP4E28iScxOg3urE1Rj49Xj4fQNiVHlBB2bGrjjhhNuVhCA0qUjAiN2TIaEELSKIn5_Rpa9_4xnJIlfBWtUZEoIRgZoOISfDMttW8Lg2uYz1xT6a-39xPsYN64Ftsg4HYGeAQeytKbmbZB7Ry-_vzIC926ImiAJ_VTUzYPBeCuzvH486PSbS_sj64n44NNtGJ16WGr3xvo7uz0djiOLiaj8-HJRWS44iLidJoqKbkQVEqTpoYlccIt5LECTWJljckhZTIJw22SC62MsTS3Shie5lO-gfYWvnPXPHfg26wKCUN0XUPT-UxIxVKieAAPF6BxjfcObDZ3RaXda0ZJ9lNs9lNs9ltsgHd6125aQf6H9k0GYLcHtDe6tE7XpvB_nJRMJZQFji64l6KE139eZsOr4c3i-TfymZLq</recordid><startdate>20060515</startdate><enddate>20060515</enddate><creator>Furtwaengler, Rhoikos</creator><creator>Reinhard, Harald</creator><creator>Leuschner, Ivo</creator><creator>Schenk, Jens P.</creator><creator>Goebel, Ulrich</creator><creator>Claviez, Alexander</creator><creator>Kulozik, Andreas</creator><creator>Zoubek, Andreas</creator><creator>von Schweinitz, Dietrich</creator><creator>Graf, Norbert</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley-Liss</general><scope>IQODW</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>7X8</scope></search><sort><creationdate>20060515</creationdate><title>Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)</title><author>Furtwaengler, Rhoikos ; Reinhard, Harald ; Leuschner, Ivo ; Schenk, Jens P. ; Goebel, Ulrich ; Claviez, Alexander ; Kulozik, Andreas ; Zoubek, Andreas ; von Schweinitz, Dietrich ; Graf, Norbert</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3936-31b8977366177c88c25453fed49ea049fccde82755553f5d6a9ccf1df96c38db3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2006</creationdate><topic>Age Factors</topic><topic>Biological and medical sciences</topic><topic>Biopsy, Needle</topic><topic>Chemotherapy, Adjuvant</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>childhood renal tumor</topic><topic>Cohort Studies</topic><topic>Female</topic><topic>Germany</topic><topic>Gesellschaft fur Pädiatrische Onkologie und Hämatologie</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>International Society of Pediatric Oncology</topic><topic>Kidney Neoplasms - diagnosis</topic><topic>Kidney Neoplasms - mortality</topic><topic>Kidney Neoplasms - therapy</topic><topic>Kidneys</topic><topic>Logistic Models</topic><topic>Male</topic><topic>Medical sciences</topic><topic>mesoblastic nephroma</topic><topic>multicenter study</topic><topic>neonate</topic><topic>Neoplasm Staging</topic><topic>Nephrectomy - methods</topic><topic>Nephrology. Urinary tract diseases</topic><topic>Nephroma, Mesoblastic - diagnosis</topic><topic>Nephroma, Mesoblastic - mortality</topic><topic>Nephroma, Mesoblastic - therapy</topic><topic>pediatric oncology</topic><topic>Pediatrics</topic><topic>prenatal diagnosis</topic><topic>Prognosis</topic><topic>Proportional Hazards Models</topic><topic>Retrospective Studies</topic><topic>Risk Assessment</topic><topic>Societies, Medical</topic><topic>Statistics, Nonparametric</topic><topic>Survival Analysis</topic><topic>Treatment Outcome</topic><topic>Tumors</topic><topic>Tumors of the urinary system</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Furtwaengler, Rhoikos</creatorcontrib><creatorcontrib>Reinhard, Harald</creatorcontrib><creatorcontrib>Leuschner, Ivo</creatorcontrib><creatorcontrib>Schenk, Jens P.</creatorcontrib><creatorcontrib>Goebel, Ulrich</creatorcontrib><creatorcontrib>Claviez, Alexander</creatorcontrib><creatorcontrib>Kulozik, Andreas</creatorcontrib><creatorcontrib>Zoubek, Andreas</creatorcontrib><creatorcontrib>von Schweinitz, Dietrich</creatorcontrib><creatorcontrib>Graf, Norbert</creatorcontrib><creatorcontrib>Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH) Nephroblastoma Study Group</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Furtwaengler, Rhoikos</au><au>Reinhard, Harald</au><au>Leuschner, Ivo</au><au>Schenk, Jens P.</au><au>Goebel, Ulrich</au><au>Claviez, Alexander</au><au>Kulozik, Andreas</au><au>Zoubek, Andreas</au><au>von Schweinitz, Dietrich</au><au>Graf, Norbert</au><aucorp>Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH) Nephroblastoma Study Group</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)</atitle><jtitle>Cancer</jtitle><addtitle>Cancer</addtitle><date>2006-05-15</date><risdate>2006</risdate><volume>106</volume><issue>10</issue><spage>2275</spage><epage>2283</epage><pages>2275-2283</pages><issn>0008-543X</issn><eissn>1097-0142</eissn><coden>CANCAR</coden><abstract>BACKGROUND
Surgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93‐01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.
METHODS
In total, 50 patients with MN were analyzed. Eleven patients were suspected antenatally of having a renal tumor. The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years.
RESULTS
Forty‐five patients underwent initial surgery. Five patients older than 6 months received preoperative chemotherapy. Twenty‐nine tumors were classic MN, and 21 tumors were cellular MN. Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 patients received no further treatment. For the entire group, event‐free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS. Three patients developed recurrent disease, and 2 of those patients died. Metastases to the brain, lung, and liver were observed in 1 patient.
CONCLUSIONS
Radical nephrectomy with accurate surgical‐pathologic staging is the standard of care for children with MN. Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy. Cancer 2006. © 2006 American Cancer Society.
The authors report on their analysis of prospectively collected data from 50 patients with a mesoblastic nephroma (MN). The findings indicated that patients who have the cellular subtype of MN in combination with Stage III disease and older age are at risk for recurrence and therefore may profit from adjuvant treatment.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>16596620</pmid><doi>10.1002/cncr.21836</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Age Factors Biological and medical sciences Biopsy, Needle Chemotherapy, Adjuvant Child Child, Preschool childhood renal tumor Cohort Studies Female Germany Gesellschaft fur Pädiatrische Onkologie und Hämatologie Humans Immunohistochemistry Infant Infant, Newborn International Society of Pediatric Oncology Kidney Neoplasms - diagnosis Kidney Neoplasms - mortality Kidney Neoplasms - therapy Kidneys Logistic Models Male Medical sciences mesoblastic nephroma multicenter study neonate Neoplasm Staging Nephrectomy - methods Nephrology. Urinary tract diseases Nephroma, Mesoblastic - diagnosis Nephroma, Mesoblastic - mortality Nephroma, Mesoblastic - therapy pediatric oncology Pediatrics prenatal diagnosis Prognosis Proportional Hazards Models Retrospective Studies Risk Assessment Societies, Medical Statistics, Nonparametric Survival Analysis Treatment Outcome Tumors Tumors of the urinary system |
title | Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH) |
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