SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors
Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods. We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST vers...
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| creator | Higham, Christine S. Steinberg, Seth M. Dombi, Eva Perry, Arie Helman, Lee J. Schuetze, Scott M. Ludwig, Joseph A. Staddon, Arthur Milhem, Mohammed M. Rushing, Daniel Jones, Robin L. Livingston, Michael Goldman, Stewart Moertel, Christopher Wagner, Lars Janhofer, David Annunziata, Christina M. Reinke, Denise Long, Lauren Viskochil, David Baker, Larry Widemann, Brigitte C. |
| description | Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods. We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%). Results. 34 NF1 (median age 33 years) and 14 sporadic (median age 40 years) MPNST patients enrolled. Five of 28 (17.9%) evaluable NF1 MPNST patients had a partial response (PR), as did 4 of 9 (44.4%) patients with sporadic MPNST. Stable disease (SD) was achieved in 22 NF1 and 4 sporadic MPNST patients. In both strata, results in the initial stages met criteria for expansion of enrollment. Only 1 additional PR was observed in the expanded NF1 stratum. Enrollment was slower than expected and the trial closed before full accrual. Conclusions. This trial was not powered to detect differences in response rates between NF1 and sporadic MPNST. While the OR rate was lower in NF1 compared to sporadic MPNST, qualitative responses were similar, and disease stabilization was achieved in most patients. |
| doi_str_mv | 10.1155/2017/8685638 |
| format | Article |
| fullrecord | <record><control><sourceid>gale_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5613633</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A546649007</galeid><airiti_id>P20151222005_201712_201810220030_201810220030_1_8_018</airiti_id><sourcerecordid>A546649007</sourcerecordid><originalsourceid>FETCH-LOGICAL-a5968-f74d5ebf9628bfdaedd02a0eef39d8e717d6cc3491f310d2ca16e43da40a601c3</originalsourceid><addsrcrecordid>eNqFkl2L1DAUhoso7rp657UEBBG0u_lq2u6FMAx-DIzr4IzgXcg0p9MsbTIm7cr8Ev-uKTPu7ogguTg5Oc95k7ycJHlO8DkhWXZBMckvClFkghUPklPCRJkSwdnDcZ_laU7495PkSQjXGGPOGX-cnNCSsEIwcpr8Wk6-TjEWl2jRqABoNkMrb1SLXI2mDXSub8Cr7Q4Zi5Zb55U2FVJWoysYvKvN2rtO9S6YgFa7LSCCJiG4yqge9JFAeqXMDaDPqjUbq2yPFuDNdqy1UcvH0rIB1TdoNXTOh6fJo1q1AZ4d4lny7cP71fRTOv_ycTadzFOVlaJI65zrDNZ1KWixrrUCrTFVGKBmpS4gJ7kWVcV4SWpGsKaVIgI404pjJTCp2Fnybq-7HdYd6ApsH18kt950yu-kU0YeV6xp5MbdyExEpxmLAq8PAt79GCD0sjOhgrZVFtwQJCkFF2VeUh7Rl3-h127wNn4vUpwSTmnO7qiNakEaW7t4bzWKyknGheAlxnmkzv9BxaWhM5WzUJt4ftTw6l5DtLrtm-DaoTfOhmPw7R6svAvBQ31rBsFynDg5Tpw8TFzEX9w38Bb-M2IReLMHGmO1-mn-Jzff08p405s7hxYRywilFON9C6FjKAgejxg-TogsZMzZb2TZ8aY</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1942142273</pqid></control><display><type>article</type><title>SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors</title><source>Wiley Online Library Open Access</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>PubMed Central</source><source>Alma/SFX Local Collection</source><source>PubMed Central Open Access</source><creator>Higham, Christine S. ; Steinberg, Seth M. ; Dombi, Eva ; Perry, Arie ; Helman, Lee J. ; Schuetze, Scott M. ; Ludwig, Joseph A. ; Staddon, Arthur ; Milhem, Mohammed M. ; Rushing, Daniel ; Jones, Robin L. ; Livingston, Michael ; Goldman, Stewart ; Moertel, Christopher ; Wagner, Lars ; Janhofer, David ; Annunziata, Christina M. ; Reinke, Denise ; Long, Lauren ; Viskochil, David ; Baker, Larry ; Widemann, Brigitte C.</creator><contributor>Eilber, Fritz C.</contributor><creatorcontrib>Higham, Christine S. ; Steinberg, Seth M. ; Dombi, Eva ; Perry, Arie ; Helman, Lee J. ; Schuetze, Scott M. ; Ludwig, Joseph A. ; Staddon, Arthur ; Milhem, Mohammed M. ; Rushing, Daniel ; Jones, Robin L. ; Livingston, Michael ; Goldman, Stewart ; Moertel, Christopher ; Wagner, Lars ; Janhofer, David ; Annunziata, Christina M. ; Reinke, Denise ; Long, Lauren ; Viskochil, David ; Baker, Larry ; Widemann, Brigitte C. ; Eilber, Fritz C.</creatorcontrib><description>Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods. We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%). Results. 34 NF1 (median age 33 years) and 14 sporadic (median age 40 years) MPNST patients enrolled. Five of 28 (17.9%) evaluable NF1 MPNST patients had a partial response (PR), as did 4 of 9 (44.4%) patients with sporadic MPNST. Stable disease (SD) was achieved in 22 NF1 and 4 sporadic MPNST patients. In both strata, results in the initial stages met criteria for expansion of enrollment. Only 1 additional PR was observed in the expanded NF1 stratum. Enrollment was slower than expected and the trial closed before full accrual. Conclusions. This trial was not powered to detect differences in response rates between NF1 and sporadic MPNST. While the OR rate was lower in NF1 compared to sporadic MPNST, qualitative responses were similar, and disease stabilization was achieved in most patients.</description><identifier>ISSN: 1357-714X</identifier><identifier>EISSN: 1369-1643</identifier><identifier>DOI: 10.1155/2017/8685638</identifier><identifier>PMID: 29138631</identifier><language>eng</language><publisher>Egypt: Hindawi Limiteds</publisher><subject>Adjuvant treatment ; Cancer ; Cancer therapies ; Chemotherapy ; Clinical Study ; Clinical trials ; Collaboration ; Comparative analysis ; Doxorubicin ; Etoposide ; Genetic disorders ; Hospitals ; Ifosfamide ; Medical research ; Metastasis ; Neurofibromatosis ; Neurological disorders ; Oncology ; Pediatrics ; Recklinghausen's disease ; Studies ; Tumors</subject><ispartof>Sarcoma, 2017, Vol.2017, p.1-8-018</ispartof><rights>Copyright © 2017 Christine S. Higham et al.</rights><rights>COPYRIGHT 2017 John Wiley & Sons, Inc.</rights><rights>Copyright © 2017 Christine S. Higham et al.; This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</rights><rights>Copyright © 2017 Christine S. Higham et al. 2017</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-a5968-f74d5ebf9628bfdaedd02a0eef39d8e717d6cc3491f310d2ca16e43da40a601c3</citedby><cites>FETCH-LOGICAL-a5968-f74d5ebf9628bfdaedd02a0eef39d8e717d6cc3491f310d2ca16e43da40a601c3</cites><orcidid>0000-0002-4618-3813 ; 0000-0002-9198-7175</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5613633/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5613633/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,881,4010,27900,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29138631$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Eilber, Fritz C.</contributor><creatorcontrib>Higham, Christine S.</creatorcontrib><creatorcontrib>Steinberg, Seth M.</creatorcontrib><creatorcontrib>Dombi, Eva</creatorcontrib><creatorcontrib>Perry, Arie</creatorcontrib><creatorcontrib>Helman, Lee J.</creatorcontrib><creatorcontrib>Schuetze, Scott M.</creatorcontrib><creatorcontrib>Ludwig, Joseph A.</creatorcontrib><creatorcontrib>Staddon, Arthur</creatorcontrib><creatorcontrib>Milhem, Mohammed M.</creatorcontrib><creatorcontrib>Rushing, Daniel</creatorcontrib><creatorcontrib>Jones, Robin L.</creatorcontrib><creatorcontrib>Livingston, Michael</creatorcontrib><creatorcontrib>Goldman, Stewart</creatorcontrib><creatorcontrib>Moertel, Christopher</creatorcontrib><creatorcontrib>Wagner, Lars</creatorcontrib><creatorcontrib>Janhofer, David</creatorcontrib><creatorcontrib>Annunziata, Christina M.</creatorcontrib><creatorcontrib>Reinke, Denise</creatorcontrib><creatorcontrib>Long, Lauren</creatorcontrib><creatorcontrib>Viskochil, David</creatorcontrib><creatorcontrib>Baker, Larry</creatorcontrib><creatorcontrib>Widemann, Brigitte C.</creatorcontrib><title>SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors</title><title>Sarcoma</title><addtitle>Sarcoma</addtitle><description>Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods. We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%). Results. 34 NF1 (median age 33 years) and 14 sporadic (median age 40 years) MPNST patients enrolled. Five of 28 (17.9%) evaluable NF1 MPNST patients had a partial response (PR), as did 4 of 9 (44.4%) patients with sporadic MPNST. Stable disease (SD) was achieved in 22 NF1 and 4 sporadic MPNST patients. In both strata, results in the initial stages met criteria for expansion of enrollment. Only 1 additional PR was observed in the expanded NF1 stratum. Enrollment was slower than expected and the trial closed before full accrual. Conclusions. This trial was not powered to detect differences in response rates between NF1 and sporadic MPNST. While the OR rate was lower in NF1 compared to sporadic MPNST, qualitative responses were similar, and disease stabilization was achieved in most patients.</description><subject>Adjuvant treatment</subject><subject>Cancer</subject><subject>Cancer therapies</subject><subject>Chemotherapy</subject><subject>Clinical Study</subject><subject>Clinical trials</subject><subject>Collaboration</subject><subject>Comparative analysis</subject><subject>Doxorubicin</subject><subject>Etoposide</subject><subject>Genetic disorders</subject><subject>Hospitals</subject><subject>Ifosfamide</subject><subject>Medical research</subject><subject>Metastasis</subject><subject>Neurofibromatosis</subject><subject>Neurological disorders</subject><subject>Oncology</subject><subject>Pediatrics</subject><subject>Recklinghausen's disease</subject><subject>Studies</subject><subject>Tumors</subject><issn>1357-714X</issn><issn>1369-1643</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><sourceid>RHX</sourceid><sourceid>8G5</sourceid><sourceid>BENPR</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNqFkl2L1DAUhoso7rp657UEBBG0u_lq2u6FMAx-DIzr4IzgXcg0p9MsbTIm7cr8Ev-uKTPu7ogguTg5Oc95k7ycJHlO8DkhWXZBMckvClFkghUPklPCRJkSwdnDcZ_laU7495PkSQjXGGPOGX-cnNCSsEIwcpr8Wk6-TjEWl2jRqABoNkMrb1SLXI2mDXSub8Cr7Q4Zi5Zb55U2FVJWoysYvKvN2rtO9S6YgFa7LSCCJiG4yqge9JFAeqXMDaDPqjUbq2yPFuDNdqy1UcvH0rIB1TdoNXTOh6fJo1q1AZ4d4lny7cP71fRTOv_ycTadzFOVlaJI65zrDNZ1KWixrrUCrTFVGKBmpS4gJ7kWVcV4SWpGsKaVIgI404pjJTCp2Fnybq-7HdYd6ApsH18kt950yu-kU0YeV6xp5MbdyExEpxmLAq8PAt79GCD0sjOhgrZVFtwQJCkFF2VeUh7Rl3-h127wNn4vUpwSTmnO7qiNakEaW7t4bzWKyknGheAlxnmkzv9BxaWhM5WzUJt4ftTw6l5DtLrtm-DaoTfOhmPw7R6svAvBQ31rBsFynDg5Tpw8TFzEX9w38Bb-M2IReLMHGmO1-mn-Jzff08p405s7hxYRywilFON9C6FjKAgejxg-TogsZMzZb2TZ8aY</recordid><startdate>2017</startdate><enddate>2017</enddate><creator>Higham, Christine S.</creator><creator>Steinberg, Seth M.</creator><creator>Dombi, Eva</creator><creator>Perry, Arie</creator><creator>Helman, Lee J.</creator><creator>Schuetze, Scott M.</creator><creator>Ludwig, Joseph A.</creator><creator>Staddon, Arthur</creator><creator>Milhem, Mohammed M.</creator><creator>Rushing, Daniel</creator><creator>Jones, Robin L.</creator><creator>Livingston, Michael</creator><creator>Goldman, Stewart</creator><creator>Moertel, Christopher</creator><creator>Wagner, Lars</creator><creator>Janhofer, David</creator><creator>Annunziata, Christina M.</creator><creator>Reinke, Denise</creator><creator>Long, Lauren</creator><creator>Viskochil, David</creator><creator>Baker, Larry</creator><creator>Widemann, Brigitte C.</creator><general>Hindawi Limiteds</general><general>Hindawi</general><general>John Wiley & Sons, Inc</general><general>Hindawi 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Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors</title><author>Higham, Christine S. ; Steinberg, Seth M. ; Dombi, Eva ; Perry, Arie ; Helman, Lee J. ; Schuetze, Scott M. ; Ludwig, Joseph A. ; Staddon, Arthur ; Milhem, Mohammed M. ; Rushing, Daniel ; Jones, Robin L. ; Livingston, Michael ; Goldman, Stewart ; Moertel, Christopher ; Wagner, Lars ; Janhofer, David ; Annunziata, Christina M. ; Reinke, Denise ; Long, Lauren ; Viskochil, David ; Baker, Larry ; Widemann, Brigitte C.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-a5968-f74d5ebf9628bfdaedd02a0eef39d8e717d6cc3491f310d2ca16e43da40a601c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Adjuvant treatment</topic><topic>Cancer</topic><topic>Cancer therapies</topic><topic>Chemotherapy</topic><topic>Clinical Study</topic><topic>Clinical trials</topic><topic>Collaboration</topic><topic>Comparative analysis</topic><topic>Doxorubicin</topic><topic>Etoposide</topic><topic>Genetic disorders</topic><topic>Hospitals</topic><topic>Ifosfamide</topic><topic>Medical research</topic><topic>Metastasis</topic><topic>Neurofibromatosis</topic><topic>Neurological disorders</topic><topic>Oncology</topic><topic>Pediatrics</topic><topic>Recklinghausen's disease</topic><topic>Studies</topic><topic>Tumors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Higham, Christine S.</creatorcontrib><creatorcontrib>Steinberg, Seth M.</creatorcontrib><creatorcontrib>Dombi, Eva</creatorcontrib><creatorcontrib>Perry, Arie</creatorcontrib><creatorcontrib>Helman, Lee J.</creatorcontrib><creatorcontrib>Schuetze, Scott M.</creatorcontrib><creatorcontrib>Ludwig, Joseph A.</creatorcontrib><creatorcontrib>Staddon, Arthur</creatorcontrib><creatorcontrib>Milhem, Mohammed M.</creatorcontrib><creatorcontrib>Rushing, Daniel</creatorcontrib><creatorcontrib>Jones, Robin L.</creatorcontrib><creatorcontrib>Livingston, Michael</creatorcontrib><creatorcontrib>Goldman, Stewart</creatorcontrib><creatorcontrib>Moertel, Christopher</creatorcontrib><creatorcontrib>Wagner, Lars</creatorcontrib><creatorcontrib>Janhofer, David</creatorcontrib><creatorcontrib>Annunziata, Christina M.</creatorcontrib><creatorcontrib>Reinke, Denise</creatorcontrib><creatorcontrib>Long, Lauren</creatorcontrib><creatorcontrib>Viskochil, David</creatorcontrib><creatorcontrib>Baker, Larry</creatorcontrib><creatorcontrib>Widemann, Brigitte C.</creatorcontrib><collection>Airiti Library</collection><collection>Hindawi Publishing Complete</collection><collection>Hindawi Publishing Subscription Journals</collection><collection>Hindawi Publishing Open Access</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Nursing & Allied Health 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J.</au><au>Schuetze, Scott M.</au><au>Ludwig, Joseph A.</au><au>Staddon, Arthur</au><au>Milhem, Mohammed M.</au><au>Rushing, Daniel</au><au>Jones, Robin L.</au><au>Livingston, Michael</au><au>Goldman, Stewart</au><au>Moertel, Christopher</au><au>Wagner, Lars</au><au>Janhofer, David</au><au>Annunziata, Christina M.</au><au>Reinke, Denise</au><au>Long, Lauren</au><au>Viskochil, David</au><au>Baker, Larry</au><au>Widemann, Brigitte C.</au><au>Eilber, Fritz C.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors</atitle><jtitle>Sarcoma</jtitle><addtitle>Sarcoma</addtitle><date>2017</date><risdate>2017</risdate><volume>2017</volume><spage>1</spage><epage>8-018</epage><pages>1-8-018</pages><issn>1357-714X</issn><eissn>1369-1643</eissn><abstract>Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods. We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%). Results. 34 NF1 (median age 33 years) and 14 sporadic (median age 40 years) MPNST patients enrolled. Five of 28 (17.9%) evaluable NF1 MPNST patients had a partial response (PR), as did 4 of 9 (44.4%) patients with sporadic MPNST. Stable disease (SD) was achieved in 22 NF1 and 4 sporadic MPNST patients. In both strata, results in the initial stages met criteria for expansion of enrollment. Only 1 additional PR was observed in the expanded NF1 stratum. Enrollment was slower than expected and the trial closed before full accrual. Conclusions. This trial was not powered to detect differences in response rates between NF1 and sporadic MPNST. While the OR rate was lower in NF1 compared to sporadic MPNST, qualitative responses were similar, and disease stabilization was achieved in most patients.</abstract><cop>Egypt</cop><pub>Hindawi Limiteds</pub><pmid>29138631</pmid><doi>10.1155/2017/8685638</doi><tpages>8</tpages><orcidid>https://orcid.org/0000-0002-4618-3813</orcidid><orcidid>https://orcid.org/0000-0002-9198-7175</orcidid><oa>free_for_read</oa></addata></record> |
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| ispartof | Sarcoma, 2017, Vol.2017, p.1-8-018 |
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| language | eng |
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| source | Wiley Online Library Open Access; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central; Alma/SFX Local Collection; PubMed Central Open Access |
| subjects | Adjuvant treatment Cancer Cancer therapies Chemotherapy Clinical Study Clinical trials Collaboration Comparative analysis Doxorubicin Etoposide Genetic disorders Hospitals Ifosfamide Medical research Metastasis Neurofibromatosis Neurological disorders Oncology Pediatrics Recklinghausen's disease Studies Tumors |
| title | SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors |
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