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Localized Childhood Soft Tissue Sarcoma

MedGen UID:
857721
•Concept ID:
C3898127
•
Neoplastic Process
Synonym: Non-Metastatic Childhood Soft Tissue Sarcoma

Definition

A soft tissue sarcoma that occurs during childhood and is confined to a specific site without evidence of spread to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocalized Childhood Soft Tissue Sarcoma

Professional guidelines

PubMed

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
An update in the management of pediatric sarcoma.
Sandler G, Yokoi A, Hayes-Jordan A
Curr Opin Pediatr 2019 Jun;31(3):368-377. doi: 10.1097/MOP.0000000000000767. PMID: 31090579
Radiotherapy in the Management of Childhood Rhabdomyosarcoma.
Mandeville HC
Clin Oncol (R Coll Radiol) 2019 Jul;31(7):462-470. Epub 2019 Apr 13 doi: 10.1016/j.clon.2019.03.047. PMID: 30992168

Recent clinical studies

Etiology

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
Osteosarcoma.
Beird HC, Bielack SS, Flanagan AM, Gill J, Heymann D, Janeway KA, Livingston JA, Roberts RD, Strauss SJ, Gorlick R
Nat Rev Dis Primers 2022 Dec 8;8(1):77. doi: 10.1038/s41572-022-00409-y. PMID: 36481668
Small round cell sarcomas.
Cidre-Aranaz F, Watson S, Amatruda JF, Nakamura T, Delattre O, de Alava E, Dirksen U, Grünewald TGP
Nat Rev Dis Primers 2022 Oct 6;8(1):66. doi: 10.1038/s41572-022-00393-3. PMID: 36202860
Ewing sarcoma.
Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H, Sorensen PH, Delattre O, Dirksen U
Nat Rev Dis Primers 2018 Jul 5;4(1):5. doi: 10.1038/s41572-018-0003-x. PMID: 29977059
The epidemiology of osteosarcoma.
Ottaviani G, Jaffe N
Cancer Treat Res 2009;152:3-13. doi: 10.1007/978-1-4419-0284-9_1. PMID: 20213383

Diagnosis

Osteosarcoma.
Beird HC, Bielack SS, Flanagan AM, Gill J, Heymann D, Janeway KA, Livingston JA, Roberts RD, Strauss SJ, Gorlick R
Nat Rev Dis Primers 2022 Dec 8;8(1):77. doi: 10.1038/s41572-022-00409-y. PMID: 36481668
Small round cell sarcomas.
Cidre-Aranaz F, Watson S, Amatruda JF, Nakamura T, Delattre O, de Alava E, Dirksen U, Grünewald TGP
Nat Rev Dis Primers 2022 Oct 6;8(1):66. doi: 10.1038/s41572-022-00393-3. PMID: 36202860
Chondroblastoma-like Osteosarcoma.
Hmada YA, Bernieh A, Morris RW, Lewin J, Allen T
Arch Pathol Lab Med 2020 Jan;144(1):15-17. Epub 2019 Aug 7 doi: 10.5858/arpa.2019-0191-RA. PMID: 31389716
Ewing sarcoma.
Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H, Sorensen PH, Delattre O, Dirksen U
Nat Rev Dis Primers 2018 Jul 5;4(1):5. doi: 10.1038/s41572-018-0003-x. PMID: 29977059
Rhabdomyosarcoma.
Dasgupta R, Fuchs J, Rodeberg D
Semin Pediatr Surg 2016 Oct;25(5):276-283. Epub 2016 Sep 20 doi: 10.1053/j.sempedsurg.2016.09.011. PMID: 27955730

Therapy

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
Fusion-negative rhabdomyosarcoma 3D organoids to predict effective drug combinations: A proof-of-concept on cell death inducers.
Savary C, Luciana L, Huchedé P, Tourbez A, Coquet C, Broustal M, Lopez Gonzalez A, Deligne C, Diot T, Naret O, Costa M, Meynard N, Barbet V, Müller K, Tonon L, Gadot N, Degletagne C, Attignon V, Léon S, Vanbelle C, Bomane A, Rochet I, Mournetas V, Oliveira L, Rinaudo P, Bergeron C, Dutour A, Cordier-Bussat M, Roch A, Brandenberg N, El Zein S, Watson S, Orbach D, Delattre O, Dijoud F, Corradini N, Picard C, Maucort-Boulch D, Le Grand M, Pasquier E, Blay JY, Castets M, Broutier L
Cell Rep Med 2023 Dec 19;4(12):101339. doi: 10.1016/j.xcrm.2023.101339. PMID: 38118405Free PMC Article
Long-Term Outcomes in Patients With Localized Ewing Sarcoma Treated With Interval-Compressed Chemotherapy on Children's Oncology Group Study AEWS0031.
Cash T, Krailo MD, Buxton AB, Pawel BR, Healey JH, Binitie O, Marcus KJ, Grier HE, Grohar PJ, Reed DR, Weiss AR, Gorlick R, Janeway KA, DuBois SG, Womer RB
J Clin Oncol 2023 Oct 20;41(30):4724-4728. Epub 2023 Aug 31 doi: 10.1200/JCO.23.00053. PMID: 37651654Free PMC Article
A Phase I/II Trial of Nivolumab plus Ipilimumab in Children and Young Adults with Relapsed/Refractory Solid Tumors: A Children's Oncology Group Study ADVL1412.
Davis KL, Fox E, Isikwei E, Reid JM, Liu X, Minard CG, Voss S, Berg SL, Weigel BJ, Mackall CL
Clin Cancer Res 2022 Dec 1;28(23):5088-5097. doi: 10.1158/1078-0432.CCR-22-2164. PMID: 36190525Free PMC Article
Nivolumab in children and young adults with relapsed or refractory solid tumours or lymphoma (ADVL1412): a multicentre, open-label, single-arm, phase 1-2 trial.
Davis KL, Fox E, Merchant MS, Reid JM, Kudgus RA, Liu X, Minard CG, Voss S, Berg SL, Weigel BJ, Mackall CL
Lancet Oncol 2020 Apr;21(4):541-550. Epub 2020 Mar 17 doi: 10.1016/S1470-2045(20)30023-1. PMID: 32192573Free PMC Article

Prognosis

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
Small round cell sarcomas.
Cidre-Aranaz F, Watson S, Amatruda JF, Nakamura T, Delattre O, de Alava E, Dirksen U, Grünewald TGP
Nat Rev Dis Primers 2022 Oct 6;8(1):66. doi: 10.1038/s41572-022-00393-3. PMID: 36202860
Chondroblastoma-like Osteosarcoma.
Hmada YA, Bernieh A, Morris RW, Lewin J, Allen T
Arch Pathol Lab Med 2020 Jan;144(1):15-17. Epub 2019 Aug 7 doi: 10.5858/arpa.2019-0191-RA. PMID: 31389716
Rhabdomyosarcoma.
Dasgupta R, Fuchs J, Rodeberg D
Semin Pediatr Surg 2016 Oct;25(5):276-283. Epub 2016 Sep 20 doi: 10.1053/j.sempedsurg.2016.09.011. PMID: 27955730
The epidemiology of osteosarcoma.
Ottaviani G, Jaffe N
Cancer Treat Res 2009;152:3-13. doi: 10.1007/978-1-4419-0284-9_1. PMID: 20213383

Clinical prediction guides

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
Fusion-negative rhabdomyosarcoma 3D organoids to predict effective drug combinations: A proof-of-concept on cell death inducers.
Savary C, Luciana L, Huchedé P, Tourbez A, Coquet C, Broustal M, Lopez Gonzalez A, Deligne C, Diot T, Naret O, Costa M, Meynard N, Barbet V, Müller K, Tonon L, Gadot N, Degletagne C, Attignon V, Léon S, Vanbelle C, Bomane A, Rochet I, Mournetas V, Oliveira L, Rinaudo P, Bergeron C, Dutour A, Cordier-Bussat M, Roch A, Brandenberg N, El Zein S, Watson S, Orbach D, Delattre O, Dijoud F, Corradini N, Picard C, Maucort-Boulch D, Le Grand M, Pasquier E, Blay JY, Castets M, Broutier L
Cell Rep Med 2023 Dec 19;4(12):101339. doi: 10.1016/j.xcrm.2023.101339. PMID: 38118405Free PMC Article
A Phase I/II Trial of Nivolumab plus Ipilimumab in Children and Young Adults with Relapsed/Refractory Solid Tumors: A Children's Oncology Group Study ADVL1412.
Davis KL, Fox E, Isikwei E, Reid JM, Liu X, Minard CG, Voss S, Berg SL, Weigel BJ, Mackall CL
Clin Cancer Res 2022 Dec 1;28(23):5088-5097. doi: 10.1158/1078-0432.CCR-22-2164. PMID: 36190525Free PMC Article
Nivolumab in children and young adults with relapsed or refractory solid tumours or lymphoma (ADVL1412): a multicentre, open-label, single-arm, phase 1-2 trial.
Davis KL, Fox E, Merchant MS, Reid JM, Kudgus RA, Liu X, Minard CG, Voss S, Berg SL, Weigel BJ, Mackall CL
Lancet Oncol 2020 Apr;21(4):541-550. Epub 2020 Mar 17 doi: 10.1016/S1470-2045(20)30023-1. PMID: 32192573Free PMC Article
Update on rhabdomyosarcoma.
Dasgupta R, Rodeberg DA
Semin Pediatr Surg 2012 Feb;21(1):68-78. doi: 10.1053/j.sempedsurg.2011.10.007. PMID: 22248972

Recent systematic reviews

A systematic review of combined surgery and brachytherapy approaches for children and young people with relapsed and refractory rhabdomyosarcoma (Local-REFoRMS).
Ballantyne E, Evans C, Shepherd L, Fulbright H, Wakeling S, Phillips B, Morgan JE
Pediatr Blood Cancer 2024 Jun;71(6):e30952. Epub 2024 Apr 2 doi: 10.1002/pbc.30952. PMID: 38566349
A systematic review of early phase studies for children and young people with relapsed and refractory rhabdomyosarcoma: The REFoRMS-SR project.
Evans C, Shepherd L, Bryan G, Fulbright H, Crowther S, Wakeling S, Stewart A, Stewart C, Chisholm J, Gibson F, Phillips B, Morgan JE
Int J Cancer 2024 Apr 1;154(7):1235-1260. Epub 2023 Dec 10 doi: 10.1002/ijc.34808. PMID: 38071594
Clinical presentation of childhood soft tissue sarcomas: a systematic review and meta-analysis.
Ni Cheallaigh L, Liu JF, Fern L, Winyard P, Walker D, Ball-Gamble A, Shanmugavadivel D
Arch Dis Child 2024 Jan 22;109(2):113-120. doi: 10.1136/archdischild-2023-325875. PMID: 37857451
Autologous hematopoietic stem cell transplantation following high-dose chemotherapy for nonrhabdomyosarcoma soft tissue sarcomas.
Peinemann F, Enk H, Smith LA
Cochrane Database Syst Rev 2017 Apr 13;4(4):CD008216. doi: 10.1002/14651858.CD008216.pub5. PMID: 28407197Free PMC Article
Current clinical evidence for proton therapy.
Brada M, Pijls-Johannesma M, De Ruysscher D
Cancer J 2009 Jul-Aug;15(4):319-24. doi: 10.1097/PPO.0b013e3181b6127c. PMID: 19672149

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