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Advanced Fibrosarcoma

MedGen UID:
1715457
•Concept ID:
C5239070
•
Neoplastic Process

Definition

Fibrosarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Fibrosarcoma

Professional guidelines

PubMed

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Advances in the Diagnosis and Management of Neonatal Sarcomas.
Rashid T, Noyd DH, Iranzad N, Davis JT, Deel MD
Clin Perinatol 2021 Mar;48(1):117-145. doi: 10.1016/j.clp.2020.11.007. PMID: 33583500
Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives.
Widemann BC, Italiano A
J Clin Oncol 2018 Jan 10;36(2):160-167. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.3467. PMID: 29220302Free PMC Article

Recent clinical studies

Etiology

Intracranial Relapse in Pediatric Sarcoma.
Smith DE, Hamby T, Heym K, Mohamed A, Vallance KL, Ray A
J Pediatr Hematol Oncol 2023 Oct 1;45(7):e810-e816. Epub 2023 Jul 14 doi: 10.1097/MPH.0000000000002713. PMID: 37526369
Dermatofibrosarcoma Protuberans: One Centre Experience.
Mareş T, Răducu L, Avino A, Gheoca-Mutu DE, Teodoreanu RN, Jecan CR
Chirurgia (Bucur) 2022 Oct;117(5):601-607. doi: 10.21614/chirurgia.2778. PMID: 36318691
Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Pan-Trk Immunohistochemistry Is an Efficient and Reliable Screen for the Detection of NTRK Fusions.
Hechtman JF, Benayed R, Hyman DM, Drilon A, Zehir A, Frosina D, Arcila ME, Dogan S, Klimstra DS, Ladanyi M, Jungbluth AA
Am J Surg Pathol 2017 Nov;41(11):1547-1551. doi: 10.1097/PAS.0000000000000911. PMID: 28719467Free PMC Article
Dermatofibrosarcoma Protuberans.
Reha J, Katz SC
Surg Clin North Am 2016 Oct;96(5):1031-46. doi: 10.1016/j.suc.2016.05.006. PMID: 27542641

Diagnosis

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives.
Widemann BC, Italiano A
J Clin Oncol 2018 Jan 10;36(2):160-167. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.3467. PMID: 29220302Free PMC Article
Dermatofibrosarcoma Protuberans.
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Pan-Trk Immunohistochemistry Is an Efficient and Reliable Screen for the Detection of NTRK Fusions.
Hechtman JF, Benayed R, Hyman DM, Drilon A, Zehir A, Frosina D, Arcila ME, Dogan S, Klimstra DS, Ladanyi M, Jungbluth AA
Am J Surg Pathol 2017 Nov;41(11):1547-1551. doi: 10.1097/PAS.0000000000000911. PMID: 28719467Free PMC Article
Dermatofibrosarcoma Protuberans.
Reha J, Katz SC
Surg Clin North Am 2016 Oct;96(5):1031-46. doi: 10.1016/j.suc.2016.05.006. PMID: 27542641

Therapy

Cancer-associated fibroblast-derived secreted phosphoprotein 1 contributes to resistance of hepatocellular carcinoma to sorafenib and lenvatinib.
Eun JW, Yoon JH, Ahn HR, Kim S, Kim YB, Lim SB, Park W, Kang TW, Baek GO, Yoon MG, Son JA, Weon JH, Kim SS, Cho HJ, Cheong JY
Cancer Commun (Lond) 2023 Apr;43(4):455-479. Epub 2023 Mar 14 doi: 10.1002/cac2.12414. PMID: 36919193Free PMC Article
Larotrectinib for paediatric solid tumours harbouring NTRK gene fusions: phase 1 results from a multicentre, open-label, phase 1/2 study.
Laetsch TW, DuBois SG, Mascarenhas L, Turpin B, Federman N, Albert CM, Nagasubramanian R, Davis JL, Rudzinski E, Feraco AM, Tuch BB, Ebata KT, Reynolds M, Smith S, Cruickshank S, Cox MC, Pappo AS, Hawkins DS
Lancet Oncol 2018 May;19(5):705-714. Epub 2018 Mar 29 doi: 10.1016/S1470-2045(18)30119-0. PMID: 29606586Free PMC Article
Dermatofibrosarcoma Protuberans.
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Leptomeningeal metastases of solid cancer.
Le Rhun E, Galanis E
Curr Opin Neurol 2016 Dec;29(6):797-805. doi: 10.1097/WCO.0000000000000393. PMID: 27661208
Current treatment options for dermatofibrosarcoma protuberans.
Rutkowski P, Debiec-Rychter M
Expert Rev Anticancer Ther 2015;15(8):901-9. Epub 2015 May 31 doi: 10.1586/14737140.2015.1052799. PMID: 26027711

Prognosis

Cancer-associated fibroblast-derived secreted phosphoprotein 1 contributes to resistance of hepatocellular carcinoma to sorafenib and lenvatinib.
Eun JW, Yoon JH, Ahn HR, Kim S, Kim YB, Lim SB, Park W, Kang TW, Baek GO, Yoon MG, Son JA, Weon JH, Kim SS, Cho HJ, Cheong JY
Cancer Commun (Lond) 2023 Apr;43(4):455-479. Epub 2023 Mar 14 doi: 10.1002/cac2.12414. PMID: 36919193Free PMC Article
CSF1/CSF1R Signaling Inhibitor Pexidartinib (PLX3397) Reprograms Tumor-Associated Macrophages and Stimulates T-cell Infiltration in the Sarcoma Microenvironment.
Fujiwara T, Yakoub MA, Chandler A, Christ AB, Yang G, Ouerfelli O, Rajasekhar VK, Yoshida A, Kondo H, Hata T, Tazawa H, Dogan Y, Moore MAS, Fujiwara T, Ozaki T, Purdue E, Healey JH
Mol Cancer Ther 2021 Aug;20(8):1388-1399. Epub 2021 Jun 4 doi: 10.1158/1535-7163.MCT-20-0591. PMID: 34088832Free PMC Article
Larotrectinib for paediatric solid tumours harbouring NTRK gene fusions: phase 1 results from a multicentre, open-label, phase 1/2 study.
Laetsch TW, DuBois SG, Mascarenhas L, Turpin B, Federman N, Albert CM, Nagasubramanian R, Davis JL, Rudzinski E, Feraco AM, Tuch BB, Ebata KT, Reynolds M, Smith S, Cruickshank S, Cox MC, Pappo AS, Hawkins DS
Lancet Oncol 2018 May;19(5):705-714. Epub 2018 Mar 29 doi: 10.1016/S1470-2045(18)30119-0. PMID: 29606586Free PMC Article
Dermatofibrosarcoma Protuberans.
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Pan-Trk Immunohistochemistry Is an Efficient and Reliable Screen for the Detection of NTRK Fusions.
Hechtman JF, Benayed R, Hyman DM, Drilon A, Zehir A, Frosina D, Arcila ME, Dogan S, Klimstra DS, Ladanyi M, Jungbluth AA
Am J Surg Pathol 2017 Nov;41(11):1547-1551. doi: 10.1097/PAS.0000000000000911. PMID: 28719467Free PMC Article

Clinical prediction guides

Cancer-associated fibroblast-derived secreted phosphoprotein 1 contributes to resistance of hepatocellular carcinoma to sorafenib and lenvatinib.
Eun JW, Yoon JH, Ahn HR, Kim S, Kim YB, Lim SB, Park W, Kang TW, Baek GO, Yoon MG, Son JA, Weon JH, Kim SS, Cho HJ, Cheong JY
Cancer Commun (Lond) 2023 Apr;43(4):455-479. Epub 2023 Mar 14 doi: 10.1002/cac2.12414. PMID: 36919193Free PMC Article
Radiosensitizing Effect of Trabectedin on Human Soft Tissue Sarcoma Cells.
Loi M, Salvatore G, Aquilano M, Greto D, Talamonti C, Salvestrini V, Melica ME, Valzano M, Francolini G, Sottili M, Santini C, Becherini C, Campanacci DA, Mangoni M, Livi L
Int J Mol Sci 2022 Nov 18;23(22) doi: 10.3390/ijms232214305. PMID: 36430780Free PMC Article
Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Larotrectinib for paediatric solid tumours harbouring NTRK gene fusions: phase 1 results from a multicentre, open-label, phase 1/2 study.
Laetsch TW, DuBois SG, Mascarenhas L, Turpin B, Federman N, Albert CM, Nagasubramanian R, Davis JL, Rudzinski E, Feraco AM, Tuch BB, Ebata KT, Reynolds M, Smith S, Cruickshank S, Cox MC, Pappo AS, Hawkins DS
Lancet Oncol 2018 May;19(5):705-714. Epub 2018 Mar 29 doi: 10.1016/S1470-2045(18)30119-0. PMID: 29606586Free PMC Article
Pan-Trk Immunohistochemistry Is an Efficient and Reliable Screen for the Detection of NTRK Fusions.
Hechtman JF, Benayed R, Hyman DM, Drilon A, Zehir A, Frosina D, Arcila ME, Dogan S, Klimstra DS, Ladanyi M, Jungbluth AA
Am J Surg Pathol 2017 Nov;41(11):1547-1551. doi: 10.1097/PAS.0000000000000911. PMID: 28719467Free PMC Article

Recent systematic reviews

Dermatofibrosarcoma protuberans of the scalp: Surgical management in a multicentric series of 11 cases and systematic review of the literature.
Kuhlmann C, Ehrl D, Taha S, Wachtel N, Schmid A, Bronsert P, Zeller J, Giunta RE, Eisenhardt SU, Braig D
Surgery 2023 Jun;173(6):1463-1475. Epub 2023 Apr 1 doi: 10.1016/j.surg.2023.02.026. PMID: 37012145
Transforming approaches to treating TRK fusion cancer: historical comparison of larotrectinib and histology-specific therapies.
Pollack M, Keating K, Wissinger E, Jackson L, Sarnes E, Cuffel B
Curr Med Res Opin 2021 Jan;37(1):59-70. Epub 2020 Nov 28 doi: 10.1080/03007995.2020.1847057. PMID: 33148054
Imatinib Treatment for Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans: A Systematic Review.
Navarrete-Dechent C, Mori S, Barker CA, Dickson MA, Nehal KS
JAMA Dermatol 2019 Mar 1;155(3):361-369. doi: 10.1001/jamadermatol.2018.4940. PMID: 30601909Free PMC Article
A systematic review of targeted agents for non-small cell lung cancer.
Vestergaard HH, Christensen MR, Lassen UN
Acta Oncol 2018 Feb;57(2):176-186. Epub 2017 Nov 25 doi: 10.1080/0284186X.2017.1404634. PMID: 29172833
A systematic review and meta-analysis of randomized trials on the role of targeted therapy in the management of advanced gastric cancer: Evidence does not translate?
Ciliberto D, Staropoli N, Caglioti F, Gualtieri S, Fiorillo L, Chiellino S, De Angelis AM, Mendicino F, Botta C, Caraglia M, Tassone P, Tagliaferri P
Cancer Biol Ther 2015;16(8):1148-59. Epub 2015 Jun 10 doi: 10.1080/15384047.2015.1056415. PMID: 26061272Free PMC Article

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