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Adult fibrosarcoma

MedGen UID:
75840
•Concept ID:
C0278595
•
Neoplastic Process
Synonyms: Adult Fibrosarcoma; adult fibrosarcoma; adult fibrosarcoma (disease); fibrosarcoma (disease) of adults
 
Monarch Initiative: MONDO:0002676

Definition

A malignant mesenchymal neoplasm composed of fibroblasts. It is characterized by collagen production and a herringbone architectural pattern. It is more commonly seen in middle-aged and older adults. It usually affects the deep soft tissues of extremities, trunk, head and neck. Adult fibrosarcomas may recur and metastasize to the lungs and bones. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult fibrosarcoma

Professional guidelines

PubMed

Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I.
Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J
Cancer Treat Rev 2021 Sep;99:102259. Epub 2021 Jul 11 doi: 10.1016/j.ctrv.2021.102259. PMID: 34311246

Recent clinical studies

Etiology

Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I.
Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J
Cancer Treat Rev 2021 Sep;99:102259. Epub 2021 Jul 11 doi: 10.1016/j.ctrv.2021.102259. PMID: 34311246
CT and MRI Findings of Soft Tissue Adult Fibrosarcoma in Extremities.
Wang H, Nie P, Dong C, Li J, Huang Y, Hao D, Xu W
Biomed Res Int 2018;2018:6075705. Epub 2018 Mar 6 doi: 10.1155/2018/6075705. PMID: 29693010Free PMC Article
Pediatric Fibroblastic and Myofibroblastic Tumors: A Pictorial Review.
Sargar KM, Sheybani EF, Shenoy A, Aranake-Chrisinger J, Khanna G
Radiographics 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191. PMID: 27399243
Adult fibrosarcoma of the wrist: a case report.
Zyluk A, Walaszek I, Grabarek J
Handchir Mikrochir Plast Chir 2012 Jun;44(3):171-4. Epub 2012 Jun 11 doi: 10.1055/s-0032-1312623. PMID: 22689150
Expression of COL1A1-PDGFB fusion transcripts in superficial adult fibrosarcoma suggests a close relationship to dermatofibrosarcoma protuberans.
Sheng WQ, Hashimoto H, Okamoto S, Ishida T, Meis-Kindblom JM, Kindblom LG, Hisaoka M
J Pathol 2001 May;194(1):88-94. doi: 10.1002/path.839. PMID: 11329146

Diagnosis

Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I.
Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J
Cancer Treat Rev 2021 Sep;99:102259. Epub 2021 Jul 11 doi: 10.1016/j.ctrv.2021.102259. PMID: 34311246
CT and MRI Findings of Soft Tissue Adult Fibrosarcoma in Extremities.
Wang H, Nie P, Dong C, Li J, Huang Y, Hao D, Xu W
Biomed Res Int 2018;2018:6075705. Epub 2018 Mar 6 doi: 10.1155/2018/6075705. PMID: 29693010Free PMC Article
Pediatric Fibroblastic and Myofibroblastic Tumors: A Pictorial Review.
Sargar KM, Sheybani EF, Shenoy A, Aranake-Chrisinger J, Khanna G
Radiographics 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191. PMID: 27399243
Fibrosarcoma: a review and update.
Folpe AL
Histopathology 2014 Jan;64(1):12-25. Epub 2013 Nov 22 doi: 10.1111/his.12282. PMID: 24266941
Infantile and adult fibrosarcomas of the soft tissues.
Iwasaki H, Enjoji M
Acta Pathol Jpn 1979 May;29(3):377-88. doi: 10.1111/j.1440-1827.1979.tb00195.x. PMID: 452901

Therapy

Sentinel node biopsy in pediatric patients: the experience in a single institution.
Dall'Igna P, De Corti F, Alaggio R, Cecchetto G
Eur J Pediatr Surg 2014 Dec;24(6):482-7. Epub 2014 Dec 5 doi: 10.1055/s-0034-1396422. PMID: 25478665

Prognosis

KIF20A promotes the development of fibrosarcoma via PI3K-Akt signaling pathway.
Jin Z, Tao S, Zhang C, Xu D, Zhu Z
Exp Cell Res 2022 Nov 1;420(1):113322. Epub 2022 Aug 28 doi: 10.1016/j.yexcr.2022.113322. PMID: 36037925
Dedifferentiated secretory breast carcinoma with fibrosarcomatous features harboring an ETV6-NTRK3 fusion in both components.
Xu J, Weisman P
Genes Chromosomes Cancer 2021 Jun;60(6):447-451. Epub 2020 Dec 28 doi: 10.1002/gcc.22929. PMID: 33342011
Pediatric Fibroblastic and Myofibroblastic Tumors: A Pictorial Review.
Sargar KM, Sheybani EF, Shenoy A, Aranake-Chrisinger J, Khanna G
Radiographics 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191. PMID: 27399243
Gains of 12q are the most frequent genomic imbalances in adult fibrosarcoma and are correlated with a poor outcome.
Schmidt H, Taubert H, Würl P, Kappler M, Lange H, Bartel F, Bache M, Holzhausen HJ, Hinze R
Genes Chromosomes Cancer 2002 May;34(1):69-77. doi: 10.1002/gcc.10036. PMID: 11921284
A comparative study of apoptosis and cell proliferation in infantile and adult fibrosarcomas.
Kihara S, Nehlsen-Cannarella N, Kirsch WM, Chase D, Garvin AJ
Am J Clin Pathol 1996 Oct;106(4):493-7. doi: 10.1093/ajcp/106.4.493. PMID: 8853038

Clinical prediction guides

Gains of 12q are the most frequent genomic imbalances in adult fibrosarcoma and are correlated with a poor outcome.
Schmidt H, Taubert H, Würl P, Kappler M, Lange H, Bartel F, Bache M, Holzhausen HJ, Hinze R
Genes Chromosomes Cancer 2002 May;34(1):69-77. doi: 10.1002/gcc.10036. PMID: 11921284
The detection of Tel-TrkC chimeric transcripts is more specific than TrkC immunoreactivity for the diagnosis of congenital fibrosarcoma.
Dubus P, Coindre JM, Groppi A, Jouan H, Ferrer J, Cohen C, Rivel J, Copin MC, Leroy JP, de Muret A, Merlio JP
J Pathol 2001 Jan;193(1):88-94. doi: 10.1002/1096-9896(2000)9999:9999<::AID-PATH724>3.0.CO;2-S. PMID: 11169520

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