Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: A report of intergroup rhabdomyosarcoma studies I through IV, 1972 through 1997
Background/Purpose: Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines....
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| Veröffentlicht in: | Journal of pediatric surgery 2003-03, Vol.38 (3), p.347-353 |
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| container_title | Journal of pediatric surgery |
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| creator | Blakely, Martin L. Andrassy, Richard J. Raney, R.Beverly Anderson, James R. Wiener, Eugene S. Rodeberg, David A. Paidas, Charles N. Lobe, Thom E Crist, William M. |
| description | Background/Purpose: Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines. Methods: From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement. Results: The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P |
| doi_str_mv | 10.1053/jpsu.2003.50106 |
| format | Article |
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This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines. Methods: From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement. Results: The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P <.001). Histology (alveolar versus embryonal) and primary site (perineal versus anal) did not significantly affect outcome. Conclusions: Because of the high incidence of regional LN involvement in these patients, a strategy of routine surgical evaluation of ilioinguinal lymph nodes in all patients with perineal or anal RMS is recommended. J Pediatr Surg 38:347-353. Copyright 2003, Elsevier Science (USA). All rights reserved.</description><identifier>ISSN: 0022-3468</identifier><identifier>EISSN: 1531-5037</identifier><identifier>DOI: 10.1053/jpsu.2003.50106</identifier><identifier>PMID: 12632347</identifier><identifier>CODEN: JPDSA3</identifier><language>eng</language><publisher>Philadelphia, PA: Elsevier Inc</publisher><subject>Adolescent ; Age Factors ; anus ; Anus Neoplasms - drug therapy ; Anus Neoplasms - mortality ; Anus Neoplasms - radiotherapy ; Anus Neoplasms - surgery ; Biological and medical sciences ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Combined Modality Therapy ; Cyclophosphamide - administration & dosage ; Dactinomycin - administration & dosage ; Disease-Free Survival ; Diseases of striated muscles. Neuromuscular diseases ; Female ; Humans ; Infant ; Infant, Newborn ; Life Tables ; lymph node biopsy ; Lymph Node Excision ; lymph node metastases ; Lymphatic Metastasis ; Male ; Medical sciences ; Multiple tumors. Solid tumors. Tumors in childhood (general aspects) ; Neoplasm Staging ; Neurology ; Perineum ; Proportional Hazards Models ; Radiotherapy, Adjuvant ; Retrospective Studies ; Rhabdomyosarcoma - drug therapy ; Rhabdomyosarcoma - mortality ; Rhabdomyosarcoma - radiotherapy ; Rhabdomyosarcoma - surgery ; Rhabdomyosarcoma of perineum ; Survival Analysis ; Survival Rate ; Treatment Outcome ; Tumors ; Vincristine - administration & dosage</subject><ispartof>Journal of pediatric surgery, 2003-03, Vol.38 (3), p.347-353</ispartof><rights>2003</rights><rights>2003 INIST-CNRS</rights><rights>Copyright 2003, Elsevier Science (USA). All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c373t-e4c02ac9cdf52ecb5780ea5cc24c672387abb16a3024be75f615f5bbcb7ae6233</citedby><cites>FETCH-LOGICAL-c373t-e4c02ac9cdf52ecb5780ea5cc24c672387abb16a3024be75f615f5bbcb7ae6233</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0022346802631148$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14585770$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12632347$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Blakely, Martin L.</creatorcontrib><creatorcontrib>Andrassy, Richard J.</creatorcontrib><creatorcontrib>Raney, R.Beverly</creatorcontrib><creatorcontrib>Anderson, James R.</creatorcontrib><creatorcontrib>Wiener, Eugene S.</creatorcontrib><creatorcontrib>Rodeberg, David A.</creatorcontrib><creatorcontrib>Paidas, Charles N.</creatorcontrib><creatorcontrib>Lobe, Thom E</creatorcontrib><creatorcontrib>Crist, William M.</creatorcontrib><creatorcontrib>Intergroup Rhabdomyosarcoma Studies I through IV</creatorcontrib><title>Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: A report of intergroup rhabdomyosarcoma studies I through IV, 1972 through 1997</title><title>Journal of pediatric surgery</title><addtitle>J Pediatr Surg</addtitle><description>Background/Purpose: Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines. Methods: From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement. Results: The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P <.001). Histology (alveolar versus embryonal) and primary site (perineal versus anal) did not significantly affect outcome. Conclusions: Because of the high incidence of regional LN involvement in these patients, a strategy of routine surgical evaluation of ilioinguinal lymph nodes in all patients with perineal or anal RMS is recommended. J Pediatr Surg 38:347-353. Copyright 2003, Elsevier Science (USA). All rights reserved.</description><subject>Adolescent</subject><subject>Age Factors</subject><subject>anus</subject><subject>Anus Neoplasms - drug therapy</subject><subject>Anus Neoplasms - mortality</subject><subject>Anus Neoplasms - radiotherapy</subject><subject>Anus Neoplasms - surgery</subject><subject>Biological and medical sciences</subject><subject>Chemotherapy, Adjuvant</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Combined Modality Therapy</subject><subject>Cyclophosphamide - administration & dosage</subject><subject>Dactinomycin - administration & dosage</subject><subject>Disease-Free Survival</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Life Tables</subject><subject>lymph node biopsy</subject><subject>Lymph Node Excision</subject><subject>lymph node metastases</subject><subject>Lymphatic Metastasis</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Multiple tumors. Solid tumors. Tumors in childhood (general aspects)</subject><subject>Neoplasm Staging</subject><subject>Neurology</subject><subject>Perineum</subject><subject>Proportional Hazards Models</subject><subject>Radiotherapy, Adjuvant</subject><subject>Retrospective Studies</subject><subject>Rhabdomyosarcoma - drug therapy</subject><subject>Rhabdomyosarcoma - mortality</subject><subject>Rhabdomyosarcoma - radiotherapy</subject><subject>Rhabdomyosarcoma - surgery</subject><subject>Rhabdomyosarcoma of perineum</subject><subject>Survival Analysis</subject><subject>Survival Rate</subject><subject>Treatment Outcome</subject><subject>Tumors</subject><subject>Vincristine - administration & dosage</subject><issn>0022-3468</issn><issn>1531-5037</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kUtv1DAUhS0EokNhzQ55w45M_YjjCbuq4jFSJVgA28i5uUlcTeLo2inqv-On4WFGdIFYXenqO-c-DmOvpdhKYfTV3RLXrRJCb42QonrCNtJoWRih7VO2EUKpQpfV7oK9iPFOZM4K-ZxdSFVppUu7Yb--UhjmEJMH3jtIgSJ3c8fjSoMHd-CJ0KUJ58SH1Xd48DNG3gfiMPpDRzjznz6NnEbXdmF6CNERhMnx0PM0Il-QsmKdeFa4eY3v-TUnXAKlI-HnhDRQWJd_DWJaO59n7bNPJoaR73-847K26m9D1rV9yZ717hDx1blesu8fP3y7-Vzcfvm0v7m-LUBbnQosQSgHNXS9UQitsTuBzgCoEiqr9M66tpWV00KVLVrTV9L0pm2htQ4rpfUluzr5AoUYCftmIT85emikaI5ZNMcsmmMWzZ8ssuLNSbGs7YTdI39-fgbengEX86t7cjP4-MiVZmesFZmrTxzm--49UhPB4wzYeUJITRf8f5f4DRkqqpk</recordid><startdate>20030301</startdate><enddate>20030301</enddate><creator>Blakely, Martin L.</creator><creator>Andrassy, Richard J.</creator><creator>Raney, R.Beverly</creator><creator>Anderson, James R.</creator><creator>Wiener, Eugene S.</creator><creator>Rodeberg, David A.</creator><creator>Paidas, Charles N.</creator><creator>Lobe, Thom E</creator><creator>Crist, William M.</creator><general>Elsevier Inc</general><general>Elsevier</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></search><sort><creationdate>20030301</creationdate><title>Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: A report of intergroup rhabdomyosarcoma studies I through IV, 1972 through 1997</title><author>Blakely, Martin L. ; Andrassy, Richard J. ; Raney, R.Beverly ; Anderson, James R. ; Wiener, Eugene S. ; Rodeberg, David A. ; Paidas, Charles N. ; Lobe, Thom E ; Crist, William M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c373t-e4c02ac9cdf52ecb5780ea5cc24c672387abb16a3024be75f615f5bbcb7ae6233</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Adolescent</topic><topic>Age Factors</topic><topic>anus</topic><topic>Anus Neoplasms - drug therapy</topic><topic>Anus Neoplasms - mortality</topic><topic>Anus Neoplasms - radiotherapy</topic><topic>Anus Neoplasms - surgery</topic><topic>Biological and medical sciences</topic><topic>Chemotherapy, Adjuvant</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Combined Modality Therapy</topic><topic>Cyclophosphamide - administration & dosage</topic><topic>Dactinomycin - administration & dosage</topic><topic>Disease-Free Survival</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>Female</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Life Tables</topic><topic>lymph node biopsy</topic><topic>Lymph Node Excision</topic><topic>lymph node metastases</topic><topic>Lymphatic Metastasis</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Multiple tumors. Solid tumors. Tumors in childhood (general aspects)</topic><topic>Neoplasm Staging</topic><topic>Neurology</topic><topic>Perineum</topic><topic>Proportional Hazards Models</topic><topic>Radiotherapy, Adjuvant</topic><topic>Retrospective Studies</topic><topic>Rhabdomyosarcoma - drug therapy</topic><topic>Rhabdomyosarcoma - mortality</topic><topic>Rhabdomyosarcoma - radiotherapy</topic><topic>Rhabdomyosarcoma - surgery</topic><topic>Rhabdomyosarcoma of perineum</topic><topic>Survival Analysis</topic><topic>Survival Rate</topic><topic>Treatment Outcome</topic><topic>Tumors</topic><topic>Vincristine - administration & dosage</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Blakely, Martin L.</creatorcontrib><creatorcontrib>Andrassy, Richard J.</creatorcontrib><creatorcontrib>Raney, R.Beverly</creatorcontrib><creatorcontrib>Anderson, James R.</creatorcontrib><creatorcontrib>Wiener, Eugene S.</creatorcontrib><creatorcontrib>Rodeberg, David A.</creatorcontrib><creatorcontrib>Paidas, Charles N.</creatorcontrib><creatorcontrib>Lobe, Thom E</creatorcontrib><creatorcontrib>Crist, William M.</creatorcontrib><creatorcontrib>Intergroup Rhabdomyosarcoma Studies I through IV</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><jtitle>Journal of pediatric surgery</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Blakely, Martin L.</au><au>Andrassy, Richard J.</au><au>Raney, R.Beverly</au><au>Anderson, James R.</au><au>Wiener, Eugene S.</au><au>Rodeberg, David A.</au><au>Paidas, Charles N.</au><au>Lobe, Thom E</au><au>Crist, William M.</au><aucorp>Intergroup Rhabdomyosarcoma Studies I through IV</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: A report of intergroup rhabdomyosarcoma studies I through IV, 1972 through 1997</atitle><jtitle>Journal of pediatric surgery</jtitle><addtitle>J Pediatr Surg</addtitle><date>2003-03-01</date><risdate>2003</risdate><volume>38</volume><issue>3</issue><spage>347</spage><epage>353</epage><pages>347-353</pages><issn>0022-3468</issn><eissn>1531-5037</eissn><coden>JPDSA3</coden><abstract>Background/Purpose: Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines. Methods: From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement. Results: The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P <.001). Histology (alveolar versus embryonal) and primary site (perineal versus anal) did not significantly affect outcome. Conclusions: Because of the high incidence of regional LN involvement in these patients, a strategy of routine surgical evaluation of ilioinguinal lymph nodes in all patients with perineal or anal RMS is recommended. J Pediatr Surg 38:347-353. Copyright 2003, Elsevier Science (USA). All rights reserved.</abstract><cop>Philadelphia, PA</cop><pub>Elsevier Inc</pub><pmid>12632347</pmid><doi>10.1053/jpsu.2003.50106</doi><tpages>7</tpages></addata></record> |
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| subjects | Adolescent Age Factors anus Anus Neoplasms - drug therapy Anus Neoplasms - mortality Anus Neoplasms - radiotherapy Anus Neoplasms - surgery Biological and medical sciences Chemotherapy, Adjuvant Child Child, Preschool Combined Modality Therapy Cyclophosphamide - administration & dosage Dactinomycin - administration & dosage Disease-Free Survival Diseases of striated muscles. Neuromuscular diseases Female Humans Infant Infant, Newborn Life Tables lymph node biopsy Lymph Node Excision lymph node metastases Lymphatic Metastasis Male Medical sciences Multiple tumors. Solid tumors. Tumors in childhood (general aspects) Neoplasm Staging Neurology Perineum Proportional Hazards Models Radiotherapy, Adjuvant Retrospective Studies Rhabdomyosarcoma - drug therapy Rhabdomyosarcoma - mortality Rhabdomyosarcoma - radiotherapy Rhabdomyosarcoma - surgery Rhabdomyosarcoma of perineum Survival Analysis Survival Rate Treatment Outcome Tumors Vincristine - administration & dosage |
| title | Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: A report of intergroup rhabdomyosarcoma studies I through IV, 1972 through 1997 |
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