U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

The Diagnosis and Treatment of Osteosarcoma and Ewing's Sarcoma in Children and Adolescents.
Zarghooni K, Bratke G, Landgraf P, Simon T, Maintz D, Eysel P
Dtsch Arztebl Int 2023 Jun 16;120(24):405-412. doi: 10.3238/arztebl.m2023.0079. PMID: 37097079Free PMC Article
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

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
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

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

Zoledronic Acid Add-on Therapy for Standard-Risk Ewing Sarcoma Patients in the Ewing 2008R1 Trial.
Koch R, Haveman L, Ladenstein R, Brichard B, Jürgens H, Cyprova S, van den Berg H, Hassenpflug W, Raciborska A, Ek T, Baumhoer D, Egerer G, Kager L, Renard M, Hauser P, Burdach S, Bovee JVMG, Hong AM, Reichardt P, Kruseova J, Streitbürger A, Kühne T, Kessler T, Bernkopf M, Butterfaß-Bahloul T, Dhooge C, Bauer S, Kiss J, Paulussen M, Bonar F, Ranft A, Timmermann B, Rascon J, Vieth V, Kanerva J, Faldum A, Hartmann W, Hjorth L, Bhadri VA, Metzler M, Gelderblom H, Dirksen U
Clin Cancer Res 2023 Dec 15;29(24):5057-5068. doi: 10.1158/1078-0432.CCR-23-1966. PMID: 37843857
Randomized Phase III Trial of Ganitumab With Interval-Compressed Chemotherapy for Patients With Newly Diagnosed Metastatic Ewing Sarcoma: A Report From the Children's Oncology Group.
DuBois SG, Krailo MD, Glade-Bender J, Buxton A, Laack N, Randall RL, Chen HX, Seibel NL, Boron M, Terezakis S, Hill-Kayser C, Hayes A, Reid JM, Teot L, Rakheja D, Womer R, Arndt C, Lessnick SL, Crompton BD, Kolb EA, Daldrup-Link H, Eutsler E, Reed DR, Janeway KA, Gorlick RG
J Clin Oncol 2023 Apr 10;41(11):2098-2107. Epub 2023 Jan 20 doi: 10.1200/JCO.22.01815. PMID: 36669140Free 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
Outcome and Patterns of Relapse in Childhood Parameningeal Rhabdomyosarcoma Treated With Proton Beam Therapy.
Doyen J, Jazmati D, Geismar D, Frisch S, Schleithoff SS, Vermeren X, Scheer M, Blase C, Tippelt S, Timmermann B
Int J Radiat Oncol Biol Phys 2019 Dec 1;105(5):1043-1054. Epub 2019 Aug 13 doi: 10.1016/j.ijrobp.2019.08.005. PMID: 31419513

Prognosis

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
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
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

Zoledronic Acid Add-on Therapy for Standard-Risk Ewing Sarcoma Patients in the Ewing 2008R1 Trial.
Koch R, Haveman L, Ladenstein R, Brichard B, Jürgens H, Cyprova S, van den Berg H, Hassenpflug W, Raciborska A, Ek T, Baumhoer D, Egerer G, Kager L, Renard M, Hauser P, Burdach S, Bovee JVMG, Hong AM, Reichardt P, Kruseova J, Streitbürger A, Kühne T, Kessler T, Bernkopf M, Butterfaß-Bahloul T, Dhooge C, Bauer S, Kiss J, Paulussen M, Bonar F, Ranft A, Timmermann B, Rascon J, Vieth V, Kanerva J, Faldum A, Hartmann W, Hjorth L, Bhadri VA, Metzler M, Gelderblom H, Dirksen U
Clin Cancer Res 2023 Dec 15;29(24):5057-5068. doi: 10.1158/1078-0432.CCR-23-1966. PMID: 37843857
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
Synovial sarcoma in children and adolescents.
Kerouanton A, Jimenez I, Cellier C, Laurence V, Helfre S, Pannier S, Mary P, Freneaux P, Orbach D
J Pediatr Hematol Oncol 2014 May;36(4):257-62. doi: 10.1097/MPH.0000000000000154. PMID: 24633301
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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...