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Metastatic Childhood Soft Tissue Sarcoma

MedGen UID:
76046
•Concept ID:
C0279942
•
Neoplastic Process
Synonyms: Metastatic Childhood Sarcoma of Soft Tissue; Metastatic Childhood Sarcoma of the Soft Tissue; Metastatic Pediatric Sarcoma of Soft Tissue; Metastatic Pediatric Sarcoma of the Soft Tissue; Metastatic Pediatric Soft Tissue Sarcoma

Definition

A sarcoma that arises from the soft tissues during childhood and has spread from the original site of growth to another anatomic site. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMetastatic 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
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
Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document.
Panda SP, Chinnaswamy G, Vora T, Prasad M, Bansal D, Kapoor G, Radhakrishnan V, Agarwala S, Laskar S, Arora B, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 May;84(5):393-402. Epub 2017 Apr 5 doi: 10.1007/s12098-017-2315-3. PMID: 28378141

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
Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.
Shern JF, Selfe J, Izquierdo E, Patidar R, Chou HC, Song YK, Yohe ME, Sindiri S, Wei J, Wen X, Rudzinski ER, Barkauskas DA, Lo T, Hall D, Linardic CM, Hughes D, Jamal S, Jenney M, Chisholm J, Brown R, Jones K, Hicks B, Angelini P, George S, Chesler L, Hubank M, Kelsey A, Gatz SA, Skapek SX, Hawkins DS, Shipley JM, Khan J
J Clin Oncol 2021 Sep 10;39(26):2859-2871. Epub 2021 Jun 24 doi: 10.1200/JCO.20.03060. PMID: 34166060Free PMC Article
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

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
Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.
Shern JF, Selfe J, Izquierdo E, Patidar R, Chou HC, Song YK, Yohe ME, Sindiri S, Wei J, Wen X, Rudzinski ER, Barkauskas DA, Lo T, Hall D, Linardic CM, Hughes D, Jamal S, Jenney M, Chisholm J, Brown R, Jones K, Hicks B, Angelini P, George S, Chesler L, Hubank M, Kelsey A, Gatz SA, Skapek SX, Hawkins DS, Shipley JM, Khan J
J Clin Oncol 2021 Sep 10;39(26):2859-2871. Epub 2021 Jun 24 doi: 10.1200/JCO.20.03060. PMID: 34166060Free PMC Article
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

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
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
High-Dose Treosulfan and Melphalan as Consolidation Therapy Versus Standard Therapy for High-Risk (Metastatic) Ewing Sarcoma.
Koch R, Gelderblom H, Haveman L, Brichard B, Jürgens H, Cyprova S, van den Berg H, Hassenpflug W, Raciborska A, Ek T, Baumhoer D, Egerer G, Eich HT, Renard M, Hauser P, Burdach S, Bovee J, Bonar F, Reichardt P, Kruseova J, Hardes J, Kühne T, Kessler T, Collaud S, Bernkopf M, Butterfaß-Bahloul T, Dhooge C, Bauer S, Kiss J, Paulussen M, Hong A, Ranft A, Timmermann B, Rascon J, Vieth V, Kanerva J, Faldum A, Metzler M, Hartmann W, Hjorth L, Bhadri V, Dirksen U
J Clin Oncol 2022 Jul 20;40(21):2307-2320. Epub 2022 Apr 15 doi: 10.1200/JCO.21.01942. PMID: 35427190
Pediatric rhabdomyosarcoma with bone marrow metastasis.
Bailey KA, Wexler LH
Pediatr Blood Cancer 2020 May;67(5):e28219. Epub 2020 Feb 26 doi: 10.1002/pbc.28219. PMID: 32100935Free PMC Article
Adjuvant and neoadjuvant chemotherapy for soft tissue sarcomas.
Maruzzo M, Rastrelli M, Lumachi F, Zagonel V, Basso U
Curr Med Chem 2013;20(5):613-20. doi: 10.2174/092986713804999385. PMID: 23278395

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
Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.
Shern JF, Selfe J, Izquierdo E, Patidar R, Chou HC, Song YK, Yohe ME, Sindiri S, Wei J, Wen X, Rudzinski ER, Barkauskas DA, Lo T, Hall D, Linardic CM, Hughes D, Jamal S, Jenney M, Chisholm J, Brown R, Jones K, Hicks B, Angelini P, George S, Chesler L, Hubank M, Kelsey A, Gatz SA, Skapek SX, Hawkins DS, Shipley JM, Khan J
J Clin Oncol 2021 Sep 10;39(26):2859-2871. Epub 2021 Jun 24 doi: 10.1200/JCO.20.03060. PMID: 34166060Free PMC Article
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
Pediatric Sarcomas.
Williams RF, Fernandez-Pineda I, Gosain A
Surg Clin North Am 2016 Oct;96(5):1107-25. doi: 10.1016/j.suc.2016.05.012. PMID: 27542645
Retinoblastoma.
Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, Brisse H, Doz F, Desjardins L
Orphanet J Rare Dis 2006 Aug 25;1:31. doi: 10.1186/1750-1172-1-31. PMID: 16934146Free PMC Article

Clinical prediction guides

High-Dose Treosulfan and Melphalan as Consolidation Therapy Versus Standard Therapy for High-Risk (Metastatic) Ewing Sarcoma.
Koch R, Gelderblom H, Haveman L, Brichard B, Jürgens H, Cyprova S, van den Berg H, Hassenpflug W, Raciborska A, Ek T, Baumhoer D, Egerer G, Eich HT, Renard M, Hauser P, Burdach S, Bovee J, Bonar F, Reichardt P, Kruseova J, Hardes J, Kühne T, Kessler T, Collaud S, Bernkopf M, Butterfaß-Bahloul T, Dhooge C, Bauer S, Kiss J, Paulussen M, Hong A, Ranft A, Timmermann B, Rascon J, Vieth V, Kanerva J, Faldum A, Metzler M, Hartmann W, Hjorth L, Bhadri V, Dirksen U
J Clin Oncol 2022 Jul 20;40(21):2307-2320. Epub 2022 Apr 15 doi: 10.1200/JCO.21.01942. PMID: 35427190
Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.
Shern JF, Selfe J, Izquierdo E, Patidar R, Chou HC, Song YK, Yohe ME, Sindiri S, Wei J, Wen X, Rudzinski ER, Barkauskas DA, Lo T, Hall D, Linardic CM, Hughes D, Jamal S, Jenney M, Chisholm J, Brown R, Jones K, Hicks B, Angelini P, George S, Chesler L, Hubank M, Kelsey A, Gatz SA, Skapek SX, Hawkins DS, Shipley JM, Khan J
J Clin Oncol 2021 Sep 10;39(26):2859-2871. Epub 2021 Jun 24 doi: 10.1200/JCO.20.03060. PMID: 34166060Free PMC Article
Subsequent neoplasms in childhood cancer survivors.
Zichová A, Eckschlager T, Ganevová M, Malinová B, Lukš A, Kruseová J
Cancer Epidemiol 2020 Oct;68:101779. Epub 2020 Jul 15 doi: 10.1016/j.canep.2020.101779. PMID: 32682319
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
Retinoblastoma.
Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, Brisse H, Doz F, Desjardins L
Orphanet J Rare Dis 2006 Aug 25;1:31. doi: 10.1186/1750-1172-1-31. PMID: 16934146Free PMC Article

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
Is perioperative chemotherapy recommended in childhood and adolescent patients with synovial sarcoma? A systematic review.
Nakamura T, Saito Y, Tsuchiya K, Miyachi M, Iwata S, Sudo A, Kawai A
Jpn J Clin Oncol 2021 May 28;51(6):927-931. doi: 10.1093/jjco/hyab039. PMID: 33822979
Target Therapies for Uterine Carcinosarcomas: Current Evidence and Future Perspectives.
Vitale SG, Laganà AS, Capriglione S, Angioli R, La Rosa VL, Lopez S, Valenti G, Sapia F, Sarpietro G, Butticè S, Tuscano C, Fanale D, Tropea A, Rossetti D
Int J Mol Sci 2017 May 20;18(5) doi: 10.3390/ijms18051100. PMID: 28531111Free PMC Article
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
An emerging evidence base for PET-CT in the management of childhood rhabdomyosarcoma: systematic review.
Norman G, Fayter D, Lewis-Light K, Chisholm J, McHugh K, Levine D, Jenney M, Mandeville H, Gatz S, Phillips B
BMJ Open 2015 Jan 8;5(1):e006030. doi: 10.1136/bmjopen-2014-006030. PMID: 25573522Free PMC Article

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