Desmoplastic small round cell tumor in children: a new therapeutic approach
Abstract Purpose Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear vis...
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| Veröffentlicht in: | Journal of pediatric surgery 2009-05, Vol.44 (5), p.949-952 |
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| description | Abstract Purpose Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. Methods Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. Results Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. Conclusion The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure. |
| doi_str_mv | 10.1016/j.jpedsurg.2009.01.071 |
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The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. Methods Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. Results Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. Conclusion The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.</description><identifier>ISSN: 0022-3468</identifier><identifier>EISSN: 1531-5037</identifier><identifier>DOI: 10.1016/j.jpedsurg.2009.01.071</identifier><identifier>PMID: 19433176</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Abdominal Neoplasms - drug therapy ; Abdominal Neoplasms - genetics ; Abdominal Neoplasms - radiotherapy ; Abdominal Neoplasms - surgery ; Abdominal Neoplasms - therapy ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Autologous bone marrow transplant ; Bone Marrow Transplantation ; Carboplatin - administration & dosage ; Child ; Chromosomes, Human, Pair 11 - genetics ; Chromosomes, Human, Pair 11 - ultrastructure ; Chromosomes, Human, Pair 22 - genetics ; Chromosomes, Human, Pair 22 - ultrastructure ; Combined Modality Therapy ; Desmoplastic small round cell tumor ; Diagnosis ; Doxorubicin - administration & dosage ; Etoposide - administration & dosage ; Female ; Humans ; Ifosfamide - administration & dosage ; Liver Neoplasms - drug therapy ; Liver Neoplasms - secondary ; Lung Neoplasms - drug therapy ; Lung Neoplasms - secondary ; Male ; Neoadjuvant Therapy ; Oncogene Proteins, Fusion - genetics ; Pathology ; Pediatrics ; Pelvic Neoplasms - drug therapy ; Pelvic Neoplasms - genetics ; Pelvic Neoplasms - radiotherapy ; Pelvic Neoplasms - surgery ; Pelvic Neoplasms - therapy ; Radiotherapy, Adjuvant ; Sarcoma, Small Cell - drug therapy ; Sarcoma, Small Cell - genetics ; Sarcoma, Small Cell - radiotherapy ; Sarcoma, Small Cell - secondary ; Sarcoma, Small Cell - surgery ; Sarcoma, Small Cell - therapy ; Splenic Neoplasms - drug therapy ; Splenic Neoplasms - secondary ; Surgery ; Translocation, Genetic ; Transplantation, Autologous ; Vincristine - administration & dosage</subject><ispartof>Journal of pediatric surgery, 2009-05, Vol.44 (5), p.949-952</ispartof><rights>Elsevier Inc.</rights><rights>2009 Elsevier Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c535t-c7bc5864cb54ee32559c905962830be9605570772779c416c7a6b048dbeece3b3</citedby><cites>FETCH-LOGICAL-c535t-c7bc5864cb54ee32559c905962830be9605570772779c416c7a6b048dbeece3b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.jpedsurg.2009.01.071$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>315,782,786,3552,27931,27932,46002</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19433176$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Al Balushi, Zainab</creatorcontrib><creatorcontrib>Bulduc, Sabrina</creatorcontrib><creatorcontrib>Mulleur, Claudia</creatorcontrib><creatorcontrib>Lallier, Michel</creatorcontrib><title>Desmoplastic small round cell tumor in children: a new therapeutic approach</title><title>Journal of pediatric surgery</title><addtitle>J Pediatr Surg</addtitle><description>Abstract Purpose Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. Methods Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. Results Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. Conclusion The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.</description><subject>Abdominal Neoplasms - drug therapy</subject><subject>Abdominal Neoplasms - genetics</subject><subject>Abdominal Neoplasms - radiotherapy</subject><subject>Abdominal Neoplasms - surgery</subject><subject>Abdominal Neoplasms - therapy</subject><subject>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</subject><subject>Autologous bone marrow transplant</subject><subject>Bone Marrow Transplantation</subject><subject>Carboplatin - administration & dosage</subject><subject>Child</subject><subject>Chromosomes, Human, Pair 11 - genetics</subject><subject>Chromosomes, Human, Pair 11 - ultrastructure</subject><subject>Chromosomes, Human, Pair 22 - genetics</subject><subject>Chromosomes, Human, Pair 22 - ultrastructure</subject><subject>Combined Modality Therapy</subject><subject>Desmoplastic small round cell tumor</subject><subject>Diagnosis</subject><subject>Doxorubicin - administration & dosage</subject><subject>Etoposide - administration & dosage</subject><subject>Female</subject><subject>Humans</subject><subject>Ifosfamide - administration & dosage</subject><subject>Liver Neoplasms - drug therapy</subject><subject>Liver Neoplasms - secondary</subject><subject>Lung Neoplasms - drug therapy</subject><subject>Lung Neoplasms - secondary</subject><subject>Male</subject><subject>Neoadjuvant Therapy</subject><subject>Oncogene Proteins, Fusion - genetics</subject><subject>Pathology</subject><subject>Pediatrics</subject><subject>Pelvic Neoplasms - drug therapy</subject><subject>Pelvic Neoplasms - genetics</subject><subject>Pelvic Neoplasms - radiotherapy</subject><subject>Pelvic Neoplasms - surgery</subject><subject>Pelvic Neoplasms - therapy</subject><subject>Radiotherapy, Adjuvant</subject><subject>Sarcoma, Small Cell - drug therapy</subject><subject>Sarcoma, Small Cell - genetics</subject><subject>Sarcoma, Small Cell - radiotherapy</subject><subject>Sarcoma, Small Cell - secondary</subject><subject>Sarcoma, Small Cell - surgery</subject><subject>Sarcoma, Small Cell - therapy</subject><subject>Splenic Neoplasms - drug therapy</subject><subject>Splenic Neoplasms - secondary</subject><subject>Surgery</subject><subject>Translocation, Genetic</subject><subject>Transplantation, Autologous</subject><subject>Vincristine - administration & dosage</subject><issn>0022-3468</issn><issn>1531-5037</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkctu1TAQhi1ERU8Lr1BlxS7pOI7tmAUCtdxEJRYtEjvLmUw5DrlhJ0V9exydg5DYsLJl_ZfxN4xdcCg4cHXZFd1MbVzD96IEMAXwAjR_wnZcCp5LEPop2wGUZS4qVZ-ysxg7gPQM_Bk75aYSgmu1Y5-vKQ7T3Lu4eMzi4Po-C9M6thlSui7rMIXMjxnufd8GGl9lLhvpV7bsKbiZ1s3l5jlMDvfP2cm96yO9OJ7n7Ov7d3dXH_ObLx8-Xb29yVEKueSoG5S1qrCRFZEopTRoQBpV1gIaMgqk1KB1qbXBiivUTjVQ1W1DhCQacc5eHnJT7c-V4mIHH7dx3UjTGq3SKVOIOgnVQYhhijHQvZ2DH1x4tBzshtF29g9Gu2G0wG3CmIwXx4a1Gaj9aztyS4I3BwGlfz54CjaipxGp9YFwse3k_9_x-p8I7P3o0fU_6JFiN61hTBQtt7G0YG-3ZW67BJP2WJtv4jfAPpv3</recordid><startdate>20090501</startdate><enddate>20090501</enddate><creator>Al Balushi, Zainab</creator><creator>Bulduc, Sabrina</creator><creator>Mulleur, Claudia</creator><creator>Lallier, Michel</creator><general>Elsevier Inc</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>7X8</scope></search><sort><creationdate>20090501</creationdate><title>Desmoplastic small round cell tumor in children: a new therapeutic approach</title><author>Al Balushi, Zainab ; Bulduc, Sabrina ; Mulleur, Claudia ; Lallier, Michel</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c535t-c7bc5864cb54ee32559c905962830be9605570772779c416c7a6b048dbeece3b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Abdominal Neoplasms - drug therapy</topic><topic>Abdominal Neoplasms - genetics</topic><topic>Abdominal Neoplasms - radiotherapy</topic><topic>Abdominal Neoplasms - surgery</topic><topic>Abdominal Neoplasms - therapy</topic><topic>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</topic><topic>Autologous bone marrow transplant</topic><topic>Bone Marrow Transplantation</topic><topic>Carboplatin - administration & dosage</topic><topic>Child</topic><topic>Chromosomes, Human, Pair 11 - genetics</topic><topic>Chromosomes, Human, Pair 11 - ultrastructure</topic><topic>Chromosomes, Human, Pair 22 - genetics</topic><topic>Chromosomes, Human, Pair 22 - ultrastructure</topic><topic>Combined Modality Therapy</topic><topic>Desmoplastic small round cell tumor</topic><topic>Diagnosis</topic><topic>Doxorubicin - administration & dosage</topic><topic>Etoposide - administration & dosage</topic><topic>Female</topic><topic>Humans</topic><topic>Ifosfamide - administration & dosage</topic><topic>Liver Neoplasms - drug therapy</topic><topic>Liver Neoplasms - secondary</topic><topic>Lung Neoplasms - drug therapy</topic><topic>Lung Neoplasms - secondary</topic><topic>Male</topic><topic>Neoadjuvant Therapy</topic><topic>Oncogene Proteins, Fusion - genetics</topic><topic>Pathology</topic><topic>Pediatrics</topic><topic>Pelvic Neoplasms - drug therapy</topic><topic>Pelvic Neoplasms - genetics</topic><topic>Pelvic Neoplasms - radiotherapy</topic><topic>Pelvic Neoplasms - surgery</topic><topic>Pelvic Neoplasms - therapy</topic><topic>Radiotherapy, Adjuvant</topic><topic>Sarcoma, Small Cell - drug therapy</topic><topic>Sarcoma, Small Cell - genetics</topic><topic>Sarcoma, Small Cell - radiotherapy</topic><topic>Sarcoma, Small Cell - secondary</topic><topic>Sarcoma, Small Cell - surgery</topic><topic>Sarcoma, Small Cell - therapy</topic><topic>Splenic Neoplasms - drug therapy</topic><topic>Splenic Neoplasms - secondary</topic><topic>Surgery</topic><topic>Translocation, Genetic</topic><topic>Transplantation, Autologous</topic><topic>Vincristine - administration & dosage</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Al Balushi, Zainab</creatorcontrib><creatorcontrib>Bulduc, Sabrina</creatorcontrib><creatorcontrib>Mulleur, Claudia</creatorcontrib><creatorcontrib>Lallier, Michel</creatorcontrib><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>Journal of pediatric surgery</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Al Balushi, Zainab</au><au>Bulduc, Sabrina</au><au>Mulleur, Claudia</au><au>Lallier, Michel</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Desmoplastic small round cell tumor in children: a new therapeutic approach</atitle><jtitle>Journal of pediatric surgery</jtitle><addtitle>J Pediatr Surg</addtitle><date>2009-05-01</date><risdate>2009</risdate><volume>44</volume><issue>5</issue><spage>949</spage><epage>952</epage><pages>949-952</pages><issn>0022-3468</issn><eissn>1531-5037</eissn><abstract>Abstract Purpose Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. Methods Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. Results Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. Conclusion The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>19433176</pmid><doi>10.1016/j.jpedsurg.2009.01.071</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Abdominal Neoplasms - drug therapy Abdominal Neoplasms - genetics Abdominal Neoplasms - radiotherapy Abdominal Neoplasms - surgery Abdominal Neoplasms - therapy Antineoplastic Combined Chemotherapy Protocols - therapeutic use Autologous bone marrow transplant Bone Marrow Transplantation Carboplatin - administration & dosage Child Chromosomes, Human, Pair 11 - genetics Chromosomes, Human, Pair 11 - ultrastructure Chromosomes, Human, Pair 22 - genetics Chromosomes, Human, Pair 22 - ultrastructure Combined Modality Therapy Desmoplastic small round cell tumor Diagnosis Doxorubicin - administration & dosage Etoposide - administration & dosage Female Humans Ifosfamide - administration & dosage Liver Neoplasms - drug therapy Liver Neoplasms - secondary Lung Neoplasms - drug therapy Lung Neoplasms - secondary Male Neoadjuvant Therapy Oncogene Proteins, Fusion - genetics Pathology Pediatrics Pelvic Neoplasms - drug therapy Pelvic Neoplasms - genetics Pelvic Neoplasms - radiotherapy Pelvic Neoplasms - surgery Pelvic Neoplasms - therapy Radiotherapy, Adjuvant Sarcoma, Small Cell - drug therapy Sarcoma, Small Cell - genetics Sarcoma, Small Cell - radiotherapy Sarcoma, Small Cell - secondary Sarcoma, Small Cell - surgery Sarcoma, Small Cell - therapy Splenic Neoplasms - drug therapy Splenic Neoplasms - secondary Surgery Translocation, Genetic Transplantation, Autologous Vincristine - administration & dosage |
| title | Desmoplastic small round cell tumor in children: a new therapeutic approach |
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