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Metastatic Unresectable Sarcoma

MedGen UID:
1657420
•Concept ID:
C4726628
•
Neoplastic Process

Definition

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

Professional guidelines

PubMed

Advances in treatment of alveolar soft part sarcoma: an updated review.
Fujiwara T, Kunisada T, Nakata E, Nishida K, Yanai H, Nakamura T, Tanaka K, Ozaki T
Jpn J Clin Oncol 2023 Nov 5;53(11):1009-1018. doi: 10.1093/jjco/hyad102. PMID: 37626447Free PMC Article
Current status of and future prospects for the treatment of unresectable or metastatic gastrointestinal stromal tumours.
Naito Y, Nishida T, Doi T
Gastric Cancer 2023 May;26(3):339-351. Epub 2023 Mar 13 doi: 10.1007/s10120-023-01381-6. PMID: 36913072Free PMC Article
Temozolomide and etoposide combination for the treatment of relapsed osteosarcoma.
Akazawa R, Umeda K, Saida S, Kato I, Hiramatsu H, Sakamoto A, Arakawa Y, Sumiyoshi S, Okamoto T, Moritake H, Adachi S, Takita J
Jpn J Clin Oncol 2020 Aug 4;50(8):948-952. doi: 10.1093/jjco/hyaa070. PMID: 32463097

Recent clinical studies

Etiology

Pembrolizumab for the adjuvant treatment of IIB or IIC melanoma.
Rutkowski P, Czarnecka AM
Expert Rev Anticancer Ther 2023 Jul-Dec;23(9):897-902. Epub 2023 Aug 13 doi: 10.1080/14737140.2023.2247565. PMID: 37573515
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
Advances in management of dermatofibrosarcoma protuberans.
Johnson-Jahangir H, Ratner D
Dermatol Clin 2011 Apr;29(2):191-200, viii. doi: 10.1016/j.det.2011.02.003. PMID: 21421145
Soft tissue sarcomas.
Lewis JJ, Brennan MF
Curr Probl Surg 1996 Oct;33(10):817-72. PMID: 8885853

Diagnosis

Afamitresgene Autoleucel: First Approval.
Keam SJ
Mol Diagn Ther 2024 Nov;28(6):861-866. Epub 2024 Oct 15 doi: 10.1007/s40291-024-00749-3. PMID: 39404764
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 Cutaneous Angiosarcoma: an Update Review.
Bi S, Zhong A, Yin X, Li J, Cen Y, Chen J
Curr Treat Options Oncol 2022 Feb;23(2):137-154. Epub 2022 Feb 19 doi: 10.1007/s11864-021-00933-1. PMID: 35182299
Soft tissue sarcomas.
Lewis JJ, Brennan MF
Curr Probl Surg 1996 Oct;33(10):817-72. PMID: 8885853

Therapy

Afamitresgene Autoleucel: First Approval.
Keam SJ
Mol Diagn Ther 2024 Nov;28(6):861-866. Epub 2024 Oct 15 doi: 10.1007/s40291-024-00749-3. PMID: 39404764
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
Management of Cutaneous Angiosarcoma: an Update Review.
Bi S, Zhong A, Yin X, Li J, Cen Y, Chen J
Curr Treat Options Oncol 2022 Feb;23(2):137-154. Epub 2022 Feb 19 doi: 10.1007/s11864-021-00933-1. PMID: 35182299
Efficacy of trabectedin for the treatment of liposarcoma.
Zijoo R, von Mehren M
Expert Opin Pharmacother 2016 Oct;17(14):1953-62. Epub 2016 Sep 15 doi: 10.1080/14656566.2016.1229304. PMID: 27615729

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
Mesenchymal Chondrosarcoma: a Review with Emphasis on its Fusion-Driven Biology.
El Beaino M, Roszik J, Livingston JA, Wang WL, Lazar AJ, Amini B, Subbiah V, Lewis V, Conley AP
Curr Oncol Rep 2018 Mar 26;20(5):37. doi: 10.1007/s11912-018-0668-z. PMID: 29582189
Advances in management of dermatofibrosarcoma protuberans.
Johnson-Jahangir H, Ratner D
Dermatol Clin 2011 Apr;29(2):191-200, viii. doi: 10.1016/j.det.2011.02.003. PMID: 21421145
Soft tissue sarcomas.
Lewis JJ, Brennan MF
Curr Probl Surg 1996 Oct;33(10):817-72. PMID: 8885853

Clinical prediction guides

A phase II study of efficacy and safety of the MEK inhibitor tunlametinib in patients with advanced NRAS-mutant melanoma.
Wei X, Zou Z, Zhang W, Fang M, Zhang X, Luo Z, Chen J, Huang G, Zhang P, Cheng Y, Liu J, Liu J, Zhang J, Wu D, Chen Y, Ma X, Pan H, Jiang R, Liu X, Ren X, Tian H, Jia Z, Guo J, Si L
Eur J Cancer 2024 May;202:114008. Epub 2024 Mar 11 doi: 10.1016/j.ejca.2024.114008. PMID: 38479118
Irreversible Electroporation Combined With Dendritic Cell-based Vaccines for the Treatment of Osteosarcoma.
Sun C, Ma X, Zhou C, Zhang Z, Guo J
Anticancer Res 2023 Aug;43(8):3389-3400. doi: 10.21873/anticanres.16514. PMID: 37500144Free PMC Article
Evaluation of MGMT Gene Methylation in Neuroendocrine Neoplasms.
Della Monica R, Cuomo M, Visconti R, di Mauro A, Buonaiuto M, Costabile D, De Riso G, Di Risi T, Guadagno E, Tafuto R, Lamia S, Ottaiano A, Cappabianca P, Del Basso de Caro ML, Tatangelo F, Hench J, Frank S, Tafuto S, Chiariotti L
Oncol Res 2022 Jan 31;28(9):837-845. Epub 2021 May 20 doi: 10.3727/096504021X16214197880808. PMID: 34016221Free PMC Article
Thrombospondin 1 Triggers Osteosarcoma Cell Metastasis and Tumor Angiogenesis.
Jian YK, Zhu HY, Wu XL, Li B
Oncol Res 2019 Feb 5;27(2):211-218. Epub 2018 Mar 14 doi: 10.3727/096504018X15208993118389. PMID: 29540257Free PMC Article
Optimal management of uterine leiomyosarcoma.
O'Cearbhaill R, Hensley ML
Expert Rev Anticancer Ther 2010 Feb;10(2):153-69. doi: 10.1586/era.09.187. PMID: 20131992

Recent systematic reviews

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
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
Role of palliative resection of the primary tumour in advanced pancreatic and small intestinal neuroendocrine tumours: A systematic review and meta-analysis.
Almond LM, Hodson J, Ford SJ, Gourevitch D, Roberts KJ, Shah T, Isaac J, Desai A
Eur J Surg Oncol 2017 Oct;43(10):1808-1815. Epub 2017 May 25 doi: 10.1016/j.ejso.2017.05.016. PMID: 28583792
KRAS mutation testing of tumours in adults with metastatic colorectal cancer: a systematic review and cost-effectiveness analysis.
Westwood M, van Asselt T, Ramaekers B, Whiting P, Joore M, Armstrong N, Noake C, Ross J, Severens J, Kleijnen J
Health Technol Assess 2014 Oct;18(62):1-132. doi: 10.3310/hta18620. PMID: 25314637Free 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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