U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Soft Tissue Sarcoma, Excluding Rhabdomyosarcoma

MedGen UID:
1664358
•Concept ID:
C4725047
•
Neoplastic Process

Definition

A finding indicating the presence of a sarcoma arising from the soft tissue, other than rhabdomyosarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSoft Tissue Sarcoma, Excluding Rhabdomyosarcoma

Professional guidelines

PubMed

Impact of Rhabdomyosarcoma Treatment Modalities by Age in a Population-Based Setting.
Ferrari A, Bernasconi A, Bergamaschi L, Botta L, Andreano A, Castaing M, Rugge M, Bisogno G, Falcini F, Sacerdote C, Tagliabue G, Michiara M, Cirilli C, Barchielli A, Filiberti RA, Vitale MF, Tumino R, Stracci F, Chiaravalli S, Casanova M, Gasparini P, Milano GM, Gatta G, Trama A
J Adolesc Young Adult Oncol 2021 Jun;10(3):309-315. Epub 2020 Jul 30 doi: 10.1089/jayao.2020.0043. PMID: 32758055
Overview of primary adult retroperitoneal tumours.
Improta L, Tzanis D, Bouhadiba T, Abdelhafidh K, Bonvalot S
Eur J Surg Oncol 2020 Sep;46(9):1573-1579. Epub 2020 May 21 doi: 10.1016/j.ejso.2020.04.054. PMID: 32600897
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article

Recent clinical studies

Etiology

Sarcoma of unknown primary: myth or reality?
Rassy E, Abou-Jaoude R, Boussios S, Assi T, Kattan J, Khaled H, Pavlidis N
J Egypt Natl Canc Inst 2022 Jun 27;34(1):27. doi: 10.1186/s43046-022-00128-1. PMID: 35754068
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Primary thoracic sarcomas.
Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH
Radiographics 2002 May-Jun;22(3):621-37. doi: 10.1148/radiographics.22.3.g02ma17621. PMID: 12006691
Childhood cancer.
Miller RW, Young JL Jr, Novakovic B
Cancer 1995 Jan 1;75(1 Suppl):395-405. doi: 10.1002/1097-0142(19950101)75:1+<395::aid-cncr2820751321>3.0.co;2-w. PMID: 8001010
Neurologic complications in pediatric solid tumors.
Weyl-Ben Arush M, Stein M, Perez-Nachum M, Dale J, Babilsky H, Zelnik N, Kuten A
Oncology 1995 Mar-Apr;52(2):89-92. doi: 10.1159/000227436. PMID: 7854781

Diagnosis

Overview of primary adult retroperitoneal tumours.
Improta L, Tzanis D, Bouhadiba T, Abdelhafidh K, Bonvalot S
Eur J Surg Oncol 2020 Sep;46(9):1573-1579. Epub 2020 May 21 doi: 10.1016/j.ejso.2020.04.054. PMID: 32600897
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Rhabdomyosarcoma subtyping by immunohistochemical assessment of myogenin: tissue array study and review of the literature.
Morgenstern DA, Rees H, Sebire NJ, Shipley J, Anderson J
Pathol Oncol Res 2008 Sep;14(3):233-8. Epub 2008 May 21 doi: 10.1007/s12253-008-9012-5. PMID: 18493875
Primary thoracic sarcomas.
Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH
Radiographics 2002 May-Jun;22(3):621-37. doi: 10.1148/radiographics.22.3.g02ma17621. PMID: 12006691
Childhood cancer.
Miller RW, Young JL Jr, Novakovic B
Cancer 1995 Jan 1;75(1 Suppl):395-405. doi: 10.1002/1097-0142(19950101)75:1+<395::aid-cncr2820751321>3.0.co;2-w. PMID: 8001010

Therapy

Addition of temsirolimus to chemotherapy in children, adolescents, and young adults with intermediate-risk rhabdomyosarcoma (ARST1431): a randomised, open-label, phase 3 trial from the Children's Oncology Group.
Gupta AA, Xue W, Harrison DJ, Hawkins DS, Dasgupta R, Wolden S, Shulkin B, Qumseya A, Routh JC, MacDonald T, Feinberg S, Crompton B, Rudzinski ER, Arnold M, Venkatramani R
Lancet Oncol 2024 Jul;25(7):912-921. doi: 10.1016/S1470-2045(24)00255-9. PMID: 38936378Free PMC Article
The Impact of Radiation Therapy in Children and Adolescents With Metastatic Rhabdomyosarcoma.
Cameron AL, Elze MC, Casanova M, Geoerger B, Gaze MN, Minard-Colin V, McHugh K, van Rijn RR, Kelsey A, Martelli H, Mandeville H, Bisogno G, Lowis S, Ronghe M, Orbach D, Guizani C, Fürst-Recktenwald S, Chisholm JC, Merks JHM; European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) and the European Innovative Therapies for Children with Cancer (ITCC) Consortium
Int J Radiat Oncol Biol Phys 2021 Nov 15;111(4):968-978. Epub 2021 Jul 1 doi: 10.1016/j.ijrobp.2021.06.031. PMID: 34217789
Radiotherapy for Adult Soft Tissue Sarcomas of the Head and Neck.
O'steen L, Saldivar B, Kharod S, Bassett B, Morris CG, Mendenhall WM
Am J Clin Oncol 2020 Sep;43(9):667-669. doi: 10.1097/COC.0000000000000729. PMID: 32889838
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Neurologic complications in pediatric solid tumors.
Weyl-Ben Arush M, Stein M, Perez-Nachum M, Dale J, Babilsky H, Zelnik N, Kuten A
Oncology 1995 Mar-Apr;52(2):89-92. doi: 10.1159/000227436. PMID: 7854781

Prognosis

Sarcoma of unknown primary: myth or reality?
Rassy E, Abou-Jaoude R, Boussios S, Assi T, Kattan J, Khaled H, Pavlidis N
J Egypt Natl Canc Inst 2022 Jun 27;34(1):27. doi: 10.1186/s43046-022-00128-1. PMID: 35754068
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Rhabdomyosarcoma subtyping by immunohistochemical assessment of myogenin: tissue array study and review of the literature.
Morgenstern DA, Rees H, Sebire NJ, Shipley J, Anderson J
Pathol Oncol Res 2008 Sep;14(3):233-8. Epub 2008 May 21 doi: 10.1007/s12253-008-9012-5. PMID: 18493875
Pure alveolar rhabdomyosarcoma of the corpus uteri: description of a case with increased serum level of CA-125.
Chiarle R, Godio L, Fusi D, Soldati T, Palestro G
Gynecol Oncol 1997 Aug;66(2):320-3. doi: 10.1006/gyno.1997.4742. PMID: 9264583
Childhood cancer.
Miller RW, Young JL Jr, Novakovic B
Cancer 1995 Jan 1;75(1 Suppl):395-405. doi: 10.1002/1097-0142(19950101)75:1+<395::aid-cncr2820751321>3.0.co;2-w. PMID: 8001010

Clinical prediction guides

Addition of temsirolimus to chemotherapy in children, adolescents, and young adults with intermediate-risk rhabdomyosarcoma (ARST1431): a randomised, open-label, phase 3 trial from the Children's Oncology Group.
Gupta AA, Xue W, Harrison DJ, Hawkins DS, Dasgupta R, Wolden S, Shulkin B, Qumseya A, Routh JC, MacDonald T, Feinberg S, Crompton B, Rudzinski ER, Arnold M, Venkatramani R
Lancet Oncol 2024 Jul;25(7):912-921. doi: 10.1016/S1470-2045(24)00255-9. PMID: 38936378Free PMC Article
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Pediatric imaging in DICER1 syndrome.
Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256
Current concepts and future perspectives in retroperitoneal soft-tissue sarcoma management.
Thomas DM, O'Sullivan B, Gronchi A
Expert Rev Anticancer Ther 2009 Aug;9(8):1145-57. doi: 10.1586/era.09.77. PMID: 19671034
Neurologic complications in pediatric solid tumors.
Weyl-Ben Arush M, Stein M, Perez-Nachum M, Dale J, Babilsky H, Zelnik N, Kuten A
Oncology 1995 Mar-Apr;52(2):89-92. doi: 10.1159/000227436. PMID: 7854781

Recent systematic reviews

Pleomorphic Rhabdomyosarcoma of the Uterus - Case Report and a Systematic Review of the Literature.
Alavi S, Eckes L, Kratschell R, Taube ET, Rosseck F, Wieners G, Sehouli J
Anticancer Res 2017 May;37(5):2509-2514. doi: 10.21873/anticanres.11592. PMID: 28476820
Autologous hematopoietic stem cell transplantation following high dose chemotherapy for non-rhabdomyosarcoma soft tissue sarcomas.
Peinemann F, Smith LA, Bartel C
Cochrane Database Syst Rev 2013 Aug 7;2013(8):CD008216. doi: 10.1002/14651858.CD008216.pub4. PMID: 23925699Free PMC Article
High-dose chemotherapy for children and young adults with stage IV rhabdomyosarcoma.
Admiraal R, van der Paardt M, Kobes J, Kremer LC, Bisogno G, Merks JH
Cochrane Database Syst Rev 2010 Dec 8;(12):CD006669. doi: 10.1002/14651858.CD006669.pub2. PMID: 21154373

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.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...