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Refractory Desmoplastic Small Round Cell Tumor

MedGen UID:
1674476
•Concept ID:
C4764226
•
Neoplastic Process

Definition

Desmoplastic small round cell tumor that is resistant to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Desmoplastic Small Round Cell Tumor

Professional guidelines

PubMed

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment - Experience from a Major Pediatric Cancer Center in Europe.
Barnbrock AE, Luckowitsch M, Schmidt S, Buus-Gehrig C, Koscher L, Becker M, Vokuhl C, Klingebiel T, Lehrnbecher T, Bochennek K
Klin Padiatr 2023 Nov;235(6):360-365. Epub 2023 Jul 11 doi: 10.1055/a-2103-2781. PMID: 37494132
Anesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.
Suchar AM, Lane J, King AC, Hayes AA, Phelps JR
Paediatr Anaesth 2023 Mar;33(3):193-200. Epub 2022 Sep 8 doi: 10.1111/pan.14551. PMID: 36052662

Recent clinical studies

Etiology

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment - Experience from a Major Pediatric Cancer Center in Europe.
Barnbrock AE, Luckowitsch M, Schmidt S, Buus-Gehrig C, Koscher L, Becker M, Vokuhl C, Klingebiel T, Lehrnbecher T, Bochennek K
Klin Padiatr 2023 Nov;235(6):360-365. Epub 2023 Jul 11 doi: 10.1055/a-2103-2781. PMID: 37494132
Anesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.
Suchar AM, Lane J, King AC, Hayes AA, Phelps JR
Paediatr Anaesth 2023 Mar;33(3):193-200. Epub 2022 Sep 8 doi: 10.1111/pan.14551. PMID: 36052662
Phase I pharmacokinetic and pharmacodynamic study of pazopanib in children with soft tissue sarcoma and other refractory solid tumors: a children's oncology group phase I consortium report.
Glade Bender JL, Lee A, Reid JM, Baruchel S, Roberts T, Voss SD, Wu B, Ahern CH, Ingle AM, Harris P, Weigel BJ, Blaney SM
J Clin Oncol 2013 Aug 20;31(24):3034-43. Epub 2013 Jul 15 doi: 10.1200/JCO.2012.47.0914. PMID: 23857966Free PMC Article
Insulin growth factor-receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with refractory Ewing's sarcoma family tumors.
Naing A, LoRusso P, Fu S, Hong DS, Anderson P, Benjamin RS, Ludwig J, Chen HX, Doyle LA, Kurzrock R
Clin Cancer Res 2012 May 1;18(9):2625-31. Epub 2012 Mar 31 doi: 10.1158/1078-0432.CCR-12-0061. PMID: 22465830Free PMC Article
A phase II study of imatinib mesylate in children with refractory or relapsed solid tumors: a Children's Oncology Group study.
Bond M, Bernstein ML, Pappo A, Schultz KR, Krailo M, Blaney SM, Adamson PC
Pediatr Blood Cancer 2008 Feb;50(2):254-8. doi: 10.1002/pbc.21132. PMID: 17262795

Diagnosis

Anesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.
Suchar AM, Lane J, King AC, Hayes AA, Phelps JR
Paediatr Anaesth 2023 Mar;33(3):193-200. Epub 2022 Sep 8 doi: 10.1111/pan.14551. PMID: 36052662
Refractory hypoglycemia in a pediatric patient with desmoplastic small round cell tumor.
Almaghraby A, Brickman WJ, Goldstein JA, Habiby RL
J Pediatr Endocrinol Metab 2018 Aug 28;31(8):947-950. doi: 10.1515/jpem-2018-0107. PMID: 30044763
Activity of trabectidin in desmoplastic small round cell tumor.
López-González A, Cantos B, Tejerina E, Provencio M
Med Oncol 2011 Dec;28 Suppl 1:S644-6. Epub 2010 Oct 7 doi: 10.1007/s12032-010-9687-9. PMID: 20924717
Desmoplastic small round cell tumors of the abdomen.
Frappaz D, Bouffet E, Dolbeau D, Bouvier R, Carrie C, Louis D, Pondarre C, Tabone E, Philip T, Brunat-Mentigny M
Cancer 1994 Mar 15;73(6):1753-6. doi: 10.1002/1097-0142(19940315)73:6<1753::aid-cncr2820730633>3.0.co;2-0. PMID: 7512439

Therapy

Anesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.
Suchar AM, Lane J, King AC, Hayes AA, Phelps JR
Paediatr Anaesth 2023 Mar;33(3):193-200. Epub 2022 Sep 8 doi: 10.1111/pan.14551. PMID: 36052662
Phase II and biomarker study of programmed cell death protein 1 inhibitor nivolumab and metronomic cyclophosphamide in paediatric relapsed/refractory solid tumours: Arm G of AcSé-ESMART, a trial of the European Innovative Therapies for Children With Cancer Consortium.
Pasqualini C, Rubino J, Brard C, Cassard L, André N, Rondof W, Scoazec JY, Marchais A, Nebchi S, Boselli L, Grivel J, Aerts I, Thebaud E, Paoletti X, Minard-Colin V, Vassal G, Geoerger B
Eur J Cancer 2021 Jun;150:53-62. Epub 2021 Apr 20 doi: 10.1016/j.ejca.2021.03.032. PMID: 33892407
Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report.
Ambar NBD, de Seixas Alves MT, Lederman HM, Abib S, Duarte AAB, Caran EM
J Med Case Rep 2019 Mar 10;13(1):77. doi: 10.1186/s13256-019-1985-z. PMID: 30851735Free PMC Article
Imatinib mesylate in desmoplastic small round cell tumors.
De Sanctis R, Bertuzzi A, Bisogno G, Carli M, Ferrari A, Comandone A, Santoro A
Future Oncol 2017 Jun;13(14):1233-1237. Epub 2017 Jun 7 doi: 10.2217/fon-2016-0305. PMID: 28589771
Human Epidermal Growth Factor Receptor 2 (HER2) -Specific Chimeric Antigen Receptor-Modified T Cells for the Immunotherapy of HER2-Positive Sarcoma.
Ahmed N, Brawley VS, Hegde M, Robertson C, Ghazi A, Gerken C, Liu E, Dakhova O, Ashoori A, Corder A, Gray T, Wu MF, Liu H, Hicks J, Rainusso N, Dotti G, Mei Z, Grilley B, Gee A, Rooney CM, Brenner MK, Heslop HE, Wels WS, Wang LL, Anderson P, Gottschalk S
J Clin Oncol 2015 May 20;33(15):1688-96. Epub 2015 Mar 23 doi: 10.1200/JCO.2014.58.0225. PMID: 25800760Free PMC Article

Prognosis

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment - Experience from a Major Pediatric Cancer Center in Europe.
Barnbrock AE, Luckowitsch M, Schmidt S, Buus-Gehrig C, Koscher L, Becker M, Vokuhl C, Klingebiel T, Lehrnbecher T, Bochennek K
Klin Padiatr 2023 Nov;235(6):360-365. Epub 2023 Jul 11 doi: 10.1055/a-2103-2781. PMID: 37494132
Refractory hypoglycemia in a pediatric patient with desmoplastic small round cell tumor.
Almaghraby A, Brickman WJ, Goldstein JA, Habiby RL
J Pediatr Endocrinol Metab 2018 Aug 28;31(8):947-950. doi: 10.1515/jpem-2018-0107. PMID: 30044763
Insulin growth factor-receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with refractory Ewing's sarcoma family tumors.
Naing A, LoRusso P, Fu S, Hong DS, Anderson P, Benjamin RS, Ludwig J, Chen HX, Doyle LA, Kurzrock R
Clin Cancer Res 2012 May 1;18(9):2625-31. Epub 2012 Mar 31 doi: 10.1158/1078-0432.CCR-12-0061. PMID: 22465830Free PMC Article
A phase II study of imatinib mesylate in children with refractory or relapsed solid tumors: a Children's Oncology Group study.
Bond M, Bernstein ML, Pappo A, Schultz KR, Krailo M, Blaney SM, Adamson PC
Pediatr Blood Cancer 2008 Feb;50(2):254-8. doi: 10.1002/pbc.21132. PMID: 17262795
Phase II study of a protracted irinotecan schedule in children with refractory or recurrent soft tissue sarcoma.
Bisogno G, Riccardi R, Ruggiero A, Arcamone G, Prete A, Surico G, Provenzi M, Bertolini P, Paolucci P, Carli M
Cancer 2006 Feb 1;106(3):703-7. doi: 10.1002/cncr.21629. PMID: 16369989

Clinical prediction guides

Phase II and biomarker study of programmed cell death protein 1 inhibitor nivolumab and metronomic cyclophosphamide in paediatric relapsed/refractory solid tumours: Arm G of AcSé-ESMART, a trial of the European Innovative Therapies for Children With Cancer Consortium.
Pasqualini C, Rubino J, Brard C, Cassard L, André N, Rondof W, Scoazec JY, Marchais A, Nebchi S, Boselli L, Grivel J, Aerts I, Thebaud E, Paoletti X, Minard-Colin V, Vassal G, Geoerger B
Eur J Cancer 2021 Jun;150:53-62. Epub 2021 Apr 20 doi: 10.1016/j.ejca.2021.03.032. PMID: 33892407
Two autopsy cases of desmoplastic small round cell tumor.
Yamamoto Y, Nishijima-Matsunobu A, Hiroshima Y, Enomoto K, Inoue M, Muto O, Suzuki M, Nanjo H, Shibata H, Omori Y
Pathol Int 2020 Mar;70(3):171-178. Epub 2020 Jan 15 doi: 10.1111/pin.12897. PMID: 31944485
Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report.
Ambar NBD, de Seixas Alves MT, Lederman HM, Abib S, Duarte AAB, Caran EM
J Med Case Rep 2019 Mar 10;13(1):77. doi: 10.1186/s13256-019-1985-z. PMID: 30851735Free PMC Article
A phase I trial and pharmacokinetic study of a 24-hour infusion of trabectedin (Yondelis®, ET-743) in children and adolescents with relapsed or refractory solid tumors.
Chuk MK, Aikin A, Whitcomb T, Widemann BC, Zannikos P, Bayever E, Balis FM, Fox E
Pediatr Blood Cancer 2012 Nov;59(5):865-9. Epub 2012 Jul 27 doi: 10.1002/pbc.24201. PMID: 22847981Free PMC Article
Phase II study of ganitumab, a fully human anti-type-1 insulin-like growth factor receptor antibody, in patients with metastatic Ewing family tumors or desmoplastic small round cell tumors.
Tap WD, Demetri G, Barnette P, Desai J, Kavan P, Tozer R, Benedetto PW, Friberg G, Deng H, McCaffery I, Leitch I, Badola S, Chang S, Zhu M, Tolcher A
J Clin Oncol 2012 May 20;30(15):1849-56. Epub 2012 Apr 16 doi: 10.1200/JCO.2011.37.2359. PMID: 22508822

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