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Unresectable Epithelioid Sarcoma

MedGen UID:
1716607
•Concept ID:
C5239547
•
Neoplastic Process

Definition

Epithelioid sarcoma that is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable Epithelioid Sarcoma

Professional guidelines

PubMed

Surgical Management of Retroperitoneal Sarcoma.
Dominguez DA, Sampath S, Agulnik M, Liang Y, Nguyen B, Trisal V, Melstrom LG, Lewis AG, Paz IB, Roberts RF, Tseng WW
Curr Oncol 2023 Apr 29;30(5):4618-4631. doi: 10.3390/curroncol30050349. PMID: 37232807Free PMC Article
Management of In-Transit Metastases.
Queiroz MM, Bertolli E, Belfort FA, Munhoz RR
Curr Oncol Rep 2022 May;24(5):573-583. Epub 2022 Feb 22 doi: 10.1007/s11912-022-01216-0. PMID: 35192119
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

Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Balleyguier C, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bardet A, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Archambaud B, Duffaud F; French Sarcoma Group
Lancet Oncol 2022 Aug;23(8):1044-1054. Epub 2022 Jul 11 doi: 10.1016/S1470-2045(22)00380-1. PMID: 35835135
Non-rhabdomyosarcoma soft-tissue sarcoma.
Milgrom SA, Million L, Mandeville H, Safwat A, Ermoian RP, Terezakis S
Pediatr Blood Cancer 2021 May;68 Suppl 2:e28279. doi: 10.1002/pbc.28279. PMID: 33818885
Osteosarcoma.
Eaton BR, Schwarz R, Vatner R, Yeh B, Claude L, Indelicato DJ, Laack N
Pediatr Blood Cancer 2021 May;68 Suppl 2:e28352. Epub 2020 Aug 11 doi: 10.1002/pbc.28352. PMID: 32779875
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial.
Judson I, Verweij J, Gelderblom H, Hartmann JT, Schöffski P, Blay JY, Kerst JM, Sufliarsky J, Whelan J, Hohenberger P, Krarup-Hansen A, Alcindor T, Marreaud S, Litière S, Hermans C, Fisher C, Hogendoorn PC, dei Tos AP, van der Graaf WT; European Organisation and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Lancet Oncol 2014 Apr;15(4):415-23. Epub 2014 Mar 5 doi: 10.1016/S1470-2045(14)70063-4. PMID: 24618336

Diagnosis

NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022.
von Mehren M, Kane JM, Riedel RF, Sicklick JK, Pollack SM, Agulnik M, Bui MM, Carr-Ascher J, Choy E, Connelly M, Dry S, Ganjoo KN, Gonzalez RJ, Holder A, Homsi J, Keedy V, Kelly CM, Kim E, Liebner D, McCarter M, McGarry SV, Mesko NW, Meyer C, Pappo AS, Parkes AM, Petersen IA, Poppe M, Schuetze S, Shabason J, Spraker MB, Zimel M, Bergman MA, Sundar H, Hang LE
J Natl Compr Canc Netw 2022 Nov;20(11):1204-1214. doi: 10.6004/jnccn.2022.0058. PMID: 36351335Free PMC Article
The myogenesis program drives clonal selection and drug resistance in rhabdomyosarcoma.
Patel AG, Chen X, Huang X, Clay MR, Komorova N, Krasin MJ, Pappo A, Tillman H, Orr BA, McEvoy J, Gordon B, Blankenship K, Reilly C, Zhou X, Norrie JL, Karlstrom A, Yu J, Wodarz D, Stewart E, Dyer MA
Dev Cell 2022 May 23;57(10):1226-1240.e8. Epub 2022 Apr 27 doi: 10.1016/j.devcel.2022.04.003. PMID: 35483358Free PMC Article
Management of dermatofibrosarcoma protuberans.
Badhey AK, Tikhtman R, Tang AL
Curr Opin Otolaryngol Head Neck Surg 2021 Aug 1;29(4):278-282. doi: 10.1097/MOO.0000000000000721. PMID: 33993132
Osteosarcoma.
Ritter J, Bielack SS
Ann Oncol 2010 Oct;21 Suppl 7:vii320-5. doi: 10.1093/annonc/mdq276. PMID: 20943636
Soft-tissue sarcoma.
Gilbert NF, Cannon CP, Lin PP, Lewis VO
J Am Acad Orthop Surg 2009 Jan;17(1):40-7. doi: 10.5435/00124635-200901000-00006. PMID: 19136426

Therapy

Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Balleyguier C, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bardet A, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Archambaud B, Duffaud F; French Sarcoma Group
Lancet Oncol 2022 Aug;23(8):1044-1054. Epub 2022 Jul 11 doi: 10.1016/S1470-2045(22)00380-1. PMID: 35835135
The myogenesis program drives clonal selection and drug resistance in rhabdomyosarcoma.
Patel AG, Chen X, Huang X, Clay MR, Komorova N, Krasin MJ, Pappo A, Tillman H, Orr BA, McEvoy J, Gordon B, Blankenship K, Reilly C, Zhou X, Norrie JL, Karlstrom A, Yu J, Wodarz D, Stewart E, Dyer MA
Dev Cell 2022 May 23;57(10):1226-1240.e8. Epub 2022 Apr 27 doi: 10.1016/j.devcel.2022.04.003. PMID: 35483358Free 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
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial.
Judson I, Verweij J, Gelderblom H, Hartmann JT, Schöffski P, Blay JY, Kerst JM, Sufliarsky J, Whelan J, Hohenberger P, Krarup-Hansen A, Alcindor T, Marreaud S, Litière S, Hermans C, Fisher C, Hogendoorn PC, dei Tos AP, van der Graaf WT; European Organisation and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Lancet Oncol 2014 Apr;15(4):415-23. Epub 2014 Mar 5 doi: 10.1016/S1470-2045(14)70063-4. PMID: 24618336

Prognosis

Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Balleyguier C, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bardet A, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Archambaud B, Duffaud F; French Sarcoma Group
Lancet Oncol 2022 Aug;23(8):1044-1054. Epub 2022 Jul 11 doi: 10.1016/S1470-2045(22)00380-1. PMID: 35835135
Osteosarcoma.
Eaton BR, Schwarz R, Vatner R, Yeh B, Claude L, Indelicato DJ, Laack N
Pediatr Blood Cancer 2021 May;68 Suppl 2:e28352. Epub 2020 Aug 11 doi: 10.1002/pbc.28352. PMID: 32779875
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Osteosarcoma.
Ritter J, Bielack SS
Ann Oncol 2010 Oct;21 Suppl 7:vii320-5. doi: 10.1093/annonc/mdq276. PMID: 20943636
Soft-tissue sarcoma.
Gilbert NF, Cannon CP, Lin PP, Lewis VO
J Am Acad Orthop Surg 2009 Jan;17(1):40-7. doi: 10.5435/00124635-200901000-00006. PMID: 19136426

Clinical prediction guides

Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
Pathological response in children and adults with large unresected intermediate-grade or high-grade soft tissue sarcoma receiving preoperative chemoradiotherapy with or without pazopanib (ARST1321): a multicentre, randomised, open-label, phase 2 trial.
Weiss AR, Chen YL, Scharschmidt TJ, Chi YY, Tian J, Black JO, Davis JL, Fanburg-Smith JC, Zambrano E, Anderson J, Arens R, Binitie O, Choy E, Davis JW, Hayes-Jordan A, Kao SC, Kayton ML, Kessel S, Lim R, Meyer WH, Million L, Okuno SH, Ostrenga A, Parisi MT, Pryma DA, Randall RL, Rosen MA, Schlapkohl M, Shulkin BL, Smith EA, Sorger JI, Terezakis S, Hawkins DS, Spunt SL, Wang D
Lancet Oncol 2020 Aug;21(8):1110-1122. Epub 2020 Jul 20 doi: 10.1016/S1470-2045(20)30325-9. PMID: 32702309Free PMC Article
Avapritinib in advanced PDGFRA D842V-mutant gastrointestinal stromal tumour (NAVIGATOR): a multicentre, open-label, phase 1 trial.
Heinrich MC, Jones RL, von Mehren M, Schöffski P, Serrano C, Kang YK, Cassier PA, Mir O, Eskens F, Tap WD, Rutkowski P, Chawla SP, Trent J, Tugnait M, Evans EK, Lauz T, Zhou T, Roche M, Wolf BB, Bauer S, George S
Lancet Oncol 2020 Jul;21(7):935-946. doi: 10.1016/S1470-2045(20)30269-2. PMID: 32615108
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
Electrochemotherapy as a New Modality in Interventional Oncology: A Review.
Probst U, Fuhrmann I, Beyer L, Wiggermann P
Technol Cancer Res Treat 2018 Jan 1;17:1533033818785329. doi: 10.1177/1533033818785329. PMID: 29986632Free PMC Article

Recent systematic reviews

Efficacy and safety of carbon ion radiotherapy for bone sarcomas: a systematic review and meta-analysis.
Dong M, Liu R, Zhang Q, Luo H, Wang D, Wang Y, Chen J, Ou Y, Wang X
Radiat Oncol 2022 Oct 25;17(1):172. doi: 10.1186/s13014-022-02089-0. PMID: 36284346Free PMC Article
Is there a place for definitive radiotherapy in the treatment of unresectable soft-tissue sarcoma? A systematic review.
Allignet B, Sunyach MP, Geets X, Waissi W
Acta Oncol 2022 Jun;61(6):720-729. Epub 2022 May 15 doi: 10.1080/0284186X.2022.2066983. PMID: 35574815
Systematic review of charged-particle therapy for chordomas and sarcomas of the mobile spine and sacrum.
Pennington Z, Ehresman J, Elsamadicy AA, Shin JH, Goodwin CR, Schwab JH, Sciubba DM
Neurosurg Focus 2021 May;50(5):E17. doi: 10.3171/2021.2.FOCUS201059. PMID: 33932924
Systemic treatments for metastatic cutaneous melanoma.
Pasquali S, Hadjinicolaou AV, Chiarion Sileni V, Rossi CR, Mocellin S
Cochrane Database Syst Rev 2018 Feb 6;2(2):CD011123. doi: 10.1002/14651858.CD011123.pub2. PMID: 29405038Free PMC Article
Systemic therapy for advanced uterine sarcoma: a systematic review of the literature.
Kanjeekal S, Chambers A, Fung MF, Verma S
Gynecol Oncol 2005 May;97(2):624-37. doi: 10.1016/j.ygyno.2005.01.041. PMID: 15863170

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