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
Hauptverfasser: Furtwaengler, Rhoikos, Reinhard, Harald, Leuschner, Ivo, Schenk, Jens P., Goebel, Ulrich, Claviez, Alexander, Kulozik, Andreas, Zoubek, Andreas, von Schweinitz, Dietrich, Graf, Norbert
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container_end_page 2283
container_issue 10
container_start_page 2275
container_title Cancer
container_volume 106
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
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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&amp;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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source Wiley Free Content; MEDLINE; Wiley Online Library Journals Frontfile Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection
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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