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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
Subclassification of pleomorphic sarcomas: How and why should we care?
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
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

Diagnosis

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
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
Subclassification of pleomorphic sarcomas: How and why should we care?
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
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

Therapy

Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma: Data of the European Soft Tissue Sarcoma Registry SoTiSaR.
Heinz AT, Schönstein A, Ebinger M, Fuchs J, Timmermann B, Seitz G, Vokuhl C, Münter M, Pajtler KW, Stegmaier S, von Kalle T, Kratz CP, Ljungman G, Juntti H, Klingebiel T, Koscielniak E, Sparber-Sauer M; Cooperative Weichteilsarkom Studiengruppe
Pediatr Blood Cancer 2024 Jan;71(1):e30707. Epub 2023 Oct 9 doi: 10.1002/pbc.30707. PMID: 37814424
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
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

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
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
Paratesticular rhabdomyosarcoma: Importance of initial therapy.
Hammond WJ, Farber BA, Price AP, Wolden SL, Heaton TE, Wexler LH, La Quaglia MP
J Pediatr Surg 2017 Feb;52(2):304-308. Epub 2016 Nov 14 doi: 10.1016/j.jpedsurg.2016.11.027. PMID: 27894767Free PMC Article
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

Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma: Data of the European Soft Tissue Sarcoma Registry SoTiSaR.
Heinz AT, Schönstein A, Ebinger M, Fuchs J, Timmermann B, Seitz G, Vokuhl C, Münter M, Pajtler KW, Stegmaier S, von Kalle T, Kratz CP, Ljungman G, Juntti H, Klingebiel T, Koscielniak E, Sparber-Sauer M; Cooperative Weichteilsarkom Studiengruppe
Pediatr Blood Cancer 2024 Jan;71(1):e30707. Epub 2023 Oct 9 doi: 10.1002/pbc.30707. PMID: 37814424
High-dose Chemotherapy Response in Adults with Relapsed/Refractory Small Round Cell Tumours.
Aykan MB, Erturk I, Acar R, Yildiran GS, Yildiz B, Karadurmus N
J Coll Physicians Surg Pak 2022 Jan;32(1):51-56. doi: 10.29271/jcpsp.2022.01.51. PMID: 34983148
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
Paratesticular rhabdomyosarcoma: Importance of initial therapy.
Hammond WJ, Farber BA, Price AP, Wolden SL, Heaton TE, Wexler LH, La Quaglia MP
J Pediatr Surg 2017 Feb;52(2):304-308. Epub 2016 Nov 14 doi: 10.1016/j.jpedsurg.2016.11.027. PMID: 27894767Free PMC Article
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

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

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