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Fibrosarcoma NCI Grade 2

MedGen UID:
272631
•Concept ID:
C1333613
•
Neoplastic Process

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibrosarcoma NCI Grade 2

Professional guidelines

PubMed

The Evolving Diagnostic and Treatment Landscape of NTRK-Fusion-Driven Pediatric Cancers.
Shulman DS, DuBois SG
Paediatr Drugs 2020 Apr;22(2):189-197. doi: 10.1007/s40272-020-00380-9. PMID: 31965543

Recent clinical studies

Etiology

ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma-A Children's Oncology Group study.
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
The Evolving Diagnostic and Treatment Landscape of NTRK-Fusion-Driven Pediatric Cancers.
Shulman DS, DuBois SG
Paediatr Drugs 2020 Apr;22(2):189-197. doi: 10.1007/s40272-020-00380-9. PMID: 31965543
Recurrent YAP1 and KMT2A Gene Rearrangements in a Subset of MUC4-negative Sclerosing Epithelioid Fibrosarcoma.
Kao YC, Lee JC, Zhang L, Sung YS, Swanson D, Hsieh TH, Liu YR, Agaram NP, Huang HY, Dickson BC, Antonescu CR
Am J Surg Pathol 2020 Mar;44(3):368-377. doi: 10.1097/PAS.0000000000001382. PMID: 31592798Free PMC Article
A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma: a pathologic and molecular study of 18 cases.
Prieto-Granada C, Zhang L, Chen HW, Sung YS, Agaram NP, Jungbluth AA, Antonescu CR
Genes Chromosomes Cancer 2015 Jan;54(1):28-38. Epub 2014 Sep 18 doi: 10.1002/gcc.22215. PMID: 25231134Free PMC Article
The enigma of myxofibrosarcoma of the extremity.
Mutter RW, Singer S, Zhang Z, Brennan MF, Alektiar KM
Cancer 2012 Jan 15;118(2):518-27. Epub 2011 Jun 29 doi: 10.1002/cncr.26296. PMID: 21717447Free PMC Article

Diagnosis

PRC2-Inactivating Mutations in Cancer Enhance Cytotoxic Response to DNMT1-Targeted Therapy via Enhanced Viral Mimicry.
Patel AJ, Warda S, Maag JLV, Misra R, Miranda-Román MA, Pachai MR, Lee CJ, Li D, Wang N, Bayshtok G, Fishinevich E, Meng Y, Wong EWP, Yan J, Giff E, Pappalardi MB, McCabe MT, Fletcher JA, Rudin CM, Chandarlapaty S, Scandura JM, Koche RP, Glass JL, Antonescu CR, Zheng D, Chen Y, Chi P
Cancer Discov 2022 Sep 2;12(9):2120-2139. doi: 10.1158/2159-8290.CD-21-1671. PMID: 35789380Free PMC Article
ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma-A Children's Oncology Group study.
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
The Evolving Diagnostic and Treatment Landscape of NTRK-Fusion-Driven Pediatric Cancers.
Shulman DS, DuBois SG
Paediatr Drugs 2020 Apr;22(2):189-197. doi: 10.1007/s40272-020-00380-9. PMID: 31965543
Recurrent YAP1 and KMT2A Gene Rearrangements in a Subset of MUC4-negative Sclerosing Epithelioid Fibrosarcoma.
Kao YC, Lee JC, Zhang L, Sung YS, Swanson D, Hsieh TH, Liu YR, Agaram NP, Huang HY, Dickson BC, Antonescu CR
Am J Surg Pathol 2020 Mar;44(3):368-377. doi: 10.1097/PAS.0000000000001382. PMID: 31592798Free PMC Article
Cell cycle and apoptosis regulatory proteins, proliferative markers, cell signaling molecules, CD209, and decorin immunoreactivity in low-grade myxofibrosarcoma and myxoma.
Cates JM, Memoli VA, Gonzalez RS
Virchows Arch 2015 Aug;467(2):211-6. Epub 2015 May 5 doi: 10.1007/s00428-015-1778-8. PMID: 25940995

Therapy

ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma-A Children's Oncology Group study.
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
The Evolving Diagnostic and Treatment Landscape of NTRK-Fusion-Driven Pediatric Cancers.
Shulman DS, DuBois SG
Paediatr Drugs 2020 Apr;22(2):189-197. doi: 10.1007/s40272-020-00380-9. PMID: 31965543
A Phase II Trial of Sorafenib and Dacarbazine for Leiomyosarcoma, Synovial Sarcoma, and Malignant Peripheral Nerve Sheath Tumors.
D'Adamo DR, Dickson MA, Keohan ML, Carvajal RD, Hensley ML, Hirst CM, Ezeoke MO, Ahn L, Qin LX, Antonescu CR, Lefkowitz RA, Maki RG, Schwartz GK, Tap WD
Oncologist 2019 Jun;24(6):857-863. Epub 2018 Aug 20 doi: 10.1634/theoncologist.2018-0160. PMID: 30126857Free PMC Article
Malignant Peripheral Nerve Sheath Tumors: A Single Institution's Experience Using Combined Surgery and Radiation Therapy.
Bishop AJ, Zagars GK, Torres KE, Bird JE, Feig BW, Guadagnolo BA
Am J Clin Oncol 2018 May;41(5):465-470. doi: 10.1097/COC.0000000000000303. PMID: 27281262Free PMC Article
Why do patients with low-grade soft tissue sarcoma die?
Canter RJ, Qin LX, Ferrone CR, Maki RG, Singer S, Brennan MF
Ann Surg Oncol 2008 Dec;15(12):3550-60. Epub 2008 Oct 1 doi: 10.1245/s10434-008-0163-0. PMID: 18830667Free PMC Article

Prognosis

TRAF7-mutated Fibromyxoid Spindle Cell Tumors Are Associated With an Aggressive Clinical Course and Harbor an Undifferentiated Sarcoma Methylation Signature: A Molecular and Clinicopathologic Study of 3 Cases.
Dermawan JK, Villafania L, Bale T, Singer S, D'Angelo SP, Tap WD, Antonescu CR
Am J Surg Pathol 2023 Feb 1;47(2):270-277. Epub 2022 Nov 3 doi: 10.1097/PAS.0000000000001997. PMID: 36395468Free PMC Article
PRC2-Inactivating Mutations in Cancer Enhance Cytotoxic Response to DNMT1-Targeted Therapy via Enhanced Viral Mimicry.
Patel AJ, Warda S, Maag JLV, Misra R, Miranda-Román MA, Pachai MR, Lee CJ, Li D, Wang N, Bayshtok G, Fishinevich E, Meng Y, Wong EWP, Yan J, Giff E, Pappalardi MB, McCabe MT, Fletcher JA, Rudin CM, Chandarlapaty S, Scandura JM, Koche RP, Glass JL, Antonescu CR, Zheng D, Chen Y, Chi P
Cancer Discov 2022 Sep 2;12(9):2120-2139. doi: 10.1158/2159-8290.CD-21-1671. PMID: 35789380Free PMC Article
Recurrent YAP1 and KMT2A Gene Rearrangements in a Subset of MUC4-negative Sclerosing Epithelioid Fibrosarcoma.
Kao YC, Lee JC, Zhang L, Sung YS, Swanson D, Hsieh TH, Liu YR, Agaram NP, Huang HY, Dickson BC, Antonescu CR
Am J Surg Pathol 2020 Mar;44(3):368-377. doi: 10.1097/PAS.0000000000001382. PMID: 31592798Free PMC Article
The enigma of myxofibrosarcoma of the extremity.
Mutter RW, Singer S, Zhang Z, Brennan MF, Alektiar KM
Cancer 2012 Jan 15;118(2):518-27. Epub 2011 Jun 29 doi: 10.1002/cncr.26296. PMID: 21717447Free PMC Article
Why do patients with low-grade soft tissue sarcoma die?
Canter RJ, Qin LX, Ferrone CR, Maki RG, Singer S, Brennan MF
Ann Surg Oncol 2008 Dec;15(12):3550-60. Epub 2008 Oct 1 doi: 10.1245/s10434-008-0163-0. PMID: 18830667Free PMC Article

Clinical prediction guides

PRC2-Inactivating Mutations in Cancer Enhance Cytotoxic Response to DNMT1-Targeted Therapy via Enhanced Viral Mimicry.
Patel AJ, Warda S, Maag JLV, Misra R, Miranda-Román MA, Pachai MR, Lee CJ, Li D, Wang N, Bayshtok G, Fishinevich E, Meng Y, Wong EWP, Yan J, Giff E, Pappalardi MB, McCabe MT, Fletcher JA, Rudin CM, Chandarlapaty S, Scandura JM, Koche RP, Glass JL, Antonescu CR, Zheng D, Chen Y, Chi P
Cancer Discov 2022 Sep 2;12(9):2120-2139. doi: 10.1158/2159-8290.CD-21-1671. PMID: 35789380Free PMC Article
The enigma of myxofibrosarcoma of the extremity.
Mutter RW, Singer S, Zhang Z, Brennan MF, Alektiar KM
Cancer 2012 Jan 15;118(2):518-27. Epub 2011 Jun 29 doi: 10.1002/cncr.26296. PMID: 21717447Free PMC Article
Why do patients with low-grade soft tissue sarcoma die?
Canter RJ, Qin LX, Ferrone CR, Maki RG, Singer S, Brennan MF
Ann Surg Oncol 2008 Dec;15(12):3550-60. Epub 2008 Oct 1 doi: 10.1245/s10434-008-0163-0. PMID: 18830667Free PMC Article
Dermatofibrosarcoma protuberans of the head and neck.
Stojadinovic A, Karpoff HM, Antonescu CR, Shah JP, Singh B, Spiro RH, Dumornay W, Shaha AR
Ann Surg Oncol 2000 Oct;7(9):696-704. doi: 10.1007/s10434-000-0696-3. PMID: 11034249
Nonrhabdomyosarcoma soft tissue sarcomas in children: is age at diagnosis an important variable?
Hayes-Jordan AA, Spunt SL, Poquette CA, Cain AM, Rao BN, Pappo AS, Shochat SJ
J Pediatr Surg 2000 Jun;35(6):948-53; discussion 953-4. doi: 10.1053/jpsu.2000.6934. PMID: 10873042

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