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Refractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

MedGen UID:
1652936
•Concept ID:
C4727144
•
Neoplastic Process

Definition

A malignant small round cell tumor with or without neural differentiation that is resistant to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

Professional guidelines

PubMed

Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience.
Büyükkapu Bay S, Kebudi R, Görgün O, Zülfikar B, Darendeliler E, Çakır FB
J Oncol Pharm Pract 2019 Sep;25(6):1343-1348. Epub 2018 Aug 6 doi: 10.1177/1078155218790798. PMID: 30080131
High-dose melphalan therapy for the treatment of children with refractory neuroblastoma and Ewing's sarcoma.
Graham-Pole J, Lazarus HM, Herzig RH, Gross S, Coccia P, Weiner R, Strandjord S
Am J Pediatr Hematol Oncol 1984 Spring;6(1):17-26. PMID: 6370012
Intensive melphalan chemotherapy and cryopreserved autologous bone marrow transplantation for the treatment of refractory cancer.
Lazarus HM, Herzig RH, Graham-Pole J, Wolff SN, Phillips GL, Strandjord S, Hurd D, Forman W, Gordon EM, Coccia P
J Clin Oncol 1983 Jun;1(6):359-67. doi: 10.1200/JCO.1983.1.6.359. PMID: 6366139

Recent clinical studies

Etiology

Phase II results from a phase I/II study to assess the safety and efficacy of weekly nab-paclitaxel in paediatric patients with recurrent or refractory solid tumours: A collaboration with the European Innovative Therapies for Children with Cancer Network.
Amoroso L, Castel V, Bisogno G, Casanova M, Marquez-Vega C, Chisholm JC, Doz F, Moreno L, Ruggiero A, Gerber NU, Fagioli F, Hingorani P, Melcón SG, Slepetis R, Chen N, le Bruchec Y, Simcock M, Vassal G
Eur J Cancer 2020 Aug;135:89-97. Epub 2020 Jun 15 doi: 10.1016/j.ejca.2020.04.031. PMID: 32554315
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
Genomic Profiling of Childhood Tumor Patient-Derived Xenograft Models to Enable Rational Clinical Trial Design.
Rokita JL, Rathi KS, Cardenas MF, Upton KA, Jayaseelan J, Cross KL, Pfeil J, Egolf LE, Way GP, Farrel A, Kendsersky NM, Patel K, Gaonkar KS, Modi A, Berko ER, Lopez G, Vaksman Z, Mayoh C, Nance J, McCoy K, Haber M, Evans K, McCalmont H, Bendak K, Böhm JW, Marshall GM, Tyrrell V, Kalletla K, Braun FK, Qi L, Du Y, Zhang H, Lindsay HB, Zhao S, Shu J, Baxter P, Morton C, Kurmashev D, Zheng S, Chen Y, Bowen J, Bryan AC, Leraas KM, Coppens SE, Doddapaneni H, Momin Z, Zhang W, Sacks GI, Hart LS, Krytska K, Mosse YP, Gatto GJ, Sanchez Y, Greene CS, Diskin SJ, Vaske OM, Haussler D, Gastier-Foster JM, Kolb EA, Gorlick R, Li XN, Reynolds CP, Kurmasheva RT, Houghton PJ, Smith MA, Lock RB, Raman P, Wheeler DA, Maris JM
Cell Rep 2019 Nov 5;29(6):1675-1689.e9. doi: 10.1016/j.celrep.2019.09.071. PMID: 31693904Free PMC Article
Phase 2 trial of pemetrexed in children and adolescents with refractory solid tumors: a Children's Oncology Group study.
Warwick AB, Malempati S, Krailo M, Melemed A, Gorlick R, Ames MM, Safgren SL, Adamson PC, Blaney SM
Pediatr Blood Cancer 2013 Feb;60(2):237-41. Epub 2012 Jun 28 doi: 10.1002/pbc.24244. PMID: 22745043Free PMC Article
Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience.
Van Winkle P, Angiolillo A, Krailo M, Cheung YK, Anderson B, Davenport V, Reaman G, Cairo MS
Pediatr Blood Cancer 2005 Apr;44(4):338-47. doi: 10.1002/pbc.20227. PMID: 15503297

Diagnosis

A phase 1 study of simvastatin in combination with topotecan and cyclophosphamide in pediatric patients with relapsed and/or refractory solid and CNS tumors.
Cash T, Jonus HC, Tsvetkova M, Beumer JH, Sadanand A, Lee JY, Henry CJ, Aguilera D, Harvey RD, Goldsmith KC
Pediatr Blood Cancer 2023 Aug;70(8):e30405. Epub 2023 May 9 doi: 10.1002/pbc.30405. PMID: 37158620
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
Genomic Profiling of Childhood Tumor Patient-Derived Xenograft Models to Enable Rational Clinical Trial Design.
Rokita JL, Rathi KS, Cardenas MF, Upton KA, Jayaseelan J, Cross KL, Pfeil J, Egolf LE, Way GP, Farrel A, Kendsersky NM, Patel K, Gaonkar KS, Modi A, Berko ER, Lopez G, Vaksman Z, Mayoh C, Nance J, McCoy K, Haber M, Evans K, McCalmont H, Bendak K, Böhm JW, Marshall GM, Tyrrell V, Kalletla K, Braun FK, Qi L, Du Y, Zhang H, Lindsay HB, Zhao S, Shu J, Baxter P, Morton C, Kurmashev D, Zheng S, Chen Y, Bowen J, Bryan AC, Leraas KM, Coppens SE, Doddapaneni H, Momin Z, Zhang W, Sacks GI, Hart LS, Krytska K, Mosse YP, Gatto GJ, Sanchez Y, Greene CS, Diskin SJ, Vaske OM, Haussler D, Gastier-Foster JM, Kolb EA, Gorlick R, Li XN, Reynolds CP, Kurmasheva RT, Houghton PJ, Smith MA, Lock RB, Raman P, Wheeler DA, Maris JM
Cell Rep 2019 Nov 5;29(6):1675-1689.e9. doi: 10.1016/j.celrep.2019.09.071. PMID: 31693904Free PMC Article
Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience.
Büyükkapu Bay S, Kebudi R, Görgün O, Zülfikar B, Darendeliler E, Çakır FB
J Oncol Pharm Pract 2019 Sep;25(6):1343-1348. Epub 2018 Aug 6 doi: 10.1177/1078155218790798. PMID: 30080131
High-dose melphalan therapy for the treatment of children with refractory neuroblastoma and Ewing's sarcoma.
Graham-Pole J, Lazarus HM, Herzig RH, Gross S, Coccia P, Weiner R, Strandjord S
Am J Pediatr Hematol Oncol 1984 Spring;6(1):17-26. PMID: 6370012

Therapy

A phase 1 study of simvastatin in combination with topotecan and cyclophosphamide in pediatric patients with relapsed and/or refractory solid and CNS tumors.
Cash T, Jonus HC, Tsvetkova M, Beumer JH, Sadanand A, Lee JY, Henry CJ, Aguilera D, Harvey RD, Goldsmith KC
Pediatr Blood Cancer 2023 Aug;70(8):e30405. Epub 2023 May 9 doi: 10.1002/pbc.30405. PMID: 37158620
A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience.
Büyükkapu Bay S, Kebudi R, Görgün O, Zülfikar B, Darendeliler E, Çakır FB
J Oncol Pharm Pract 2019 Sep;25(6):1343-1348. Epub 2018 Aug 6 doi: 10.1177/1078155218790798. PMID: 30080131
Temozolomide in resistant or relapsed pediatric solid tumors.
De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A
Pediatr Blood Cancer 2006 Jul;47(1):30-6. doi: 10.1002/pbc.20516. PMID: 16047361

Prognosis

Immune contexture of paediatric cancers.
Thakur MD, Franz CJ, Brennan L, Brouwer-Visser J, Tam R, Korski K, Koeppen H, Ziai J, Babitzki G, Ranchere-Vince D, Vasiljevic A, Dijoud F, Marec-Bérard P, Rochet I, Cannarile MA, Marabelle A
Eur J Cancer 2022 Jul;170:179-193. Epub 2022 May 31 doi: 10.1016/j.ejca.2022.03.012. PMID: 35660252
Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience.
Büyükkapu Bay S, Kebudi R, Görgün O, Zülfikar B, Darendeliler E, Çakır FB
J Oncol Pharm Pract 2019 Sep;25(6):1343-1348. Epub 2018 Aug 6 doi: 10.1177/1078155218790798. PMID: 30080131
Temozolomide in resistant or relapsed pediatric solid tumors.
De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A
Pediatr Blood Cancer 2006 Jul;47(1):30-6. doi: 10.1002/pbc.20516. PMID: 16047361
Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience.
Van Winkle P, Angiolillo A, Krailo M, Cheung YK, Anderson B, Davenport V, Reaman G, Cairo MS
Pediatr Blood Cancer 2005 Apr;44(4):338-47. doi: 10.1002/pbc.20227. PMID: 15503297
High-dose melphalan therapy for the treatment of children with refractory neuroblastoma and Ewing's sarcoma.
Graham-Pole J, Lazarus HM, Herzig RH, Gross S, Coccia P, Weiner R, Strandjord S
Am J Pediatr Hematol Oncol 1984 Spring;6(1):17-26. PMID: 6370012

Clinical prediction guides

A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
Phase II results from a phase I/II study to assess the safety and efficacy of weekly nab-paclitaxel in paediatric patients with recurrent or refractory solid tumours: A collaboration with the European Innovative Therapies for Children with Cancer Network.
Amoroso L, Castel V, Bisogno G, Casanova M, Marquez-Vega C, Chisholm JC, Doz F, Moreno L, Ruggiero A, Gerber NU, Fagioli F, Hingorani P, Melcón SG, Slepetis R, Chen N, le Bruchec Y, Simcock M, Vassal G
Eur J Cancer 2020 Aug;135:89-97. Epub 2020 Jun 15 doi: 10.1016/j.ejca.2020.04.031. PMID: 32554315
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
Immunotherapy with autologous dendritic cells and tumor antigens for children with refractory malignant solid tumors.
Suminoe A, Matsuzaki A, Hattori H, Koga Y, Hara T
Pediatr Transplant 2009 Sep;13(6):746-53. Epub 2008 Nov 1 doi: 10.1111/j.1399-3046.2008.01066.x. PMID: 19067917
Temozolomide in resistant or relapsed pediatric solid tumors.
De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A
Pediatr Blood Cancer 2006 Jul;47(1):30-6. doi: 10.1002/pbc.20516. PMID: 16047361

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