Myxoid liposarcoma
| Myxoid liposarcoma | |
|---|---|
 | |
| Histopathologic image of myxoid liposarcoma arising in the thigh. H & E stain. | |
| Specialty | Oncology |
Amyxoid liposarcomais amalignantadipose tissue neoplasm[1]ofmyxoidappearance histologically.
Myxoid liposarcomas are the second-most common type ofliposarcoma,representing 30–40% of all liposarcomas in the limbs, occurring most commonly in the legs, particularly the thigh, followed by the buttocks, retroperitoneum, trunk, ankle, proximal limb girdle, head and neck, and wrist. They occur in the intermuscularfascial planesor deep-seated areas. They present as a large, slow-growing, painless mass.[2]
The neoplastic cells in these neoplasms containchromosomal translocationswhich create one of twofusion genes:theFUS-DDIT3in ~90% and theEWSR1-DDIT3fusion gene in up to 10% of myxoid liposarcoma cases.[3]TheFUS-DDIT3fusion gene forms by a merger of part of theFUSFET gene familygene normally located atband11.2 on theshort (or "p" ) armofchromosome 16with part of theDDIT3ETS transcription factor familygene normally located at band 13.3 on the long (or "q" ) arm ofchromosome 12.This fusion gene is notated as t(12;16)(q13;p11).[4][5]Preclinical studies,i.e. laboratory studies, suggest that theFUS-DDIT3fusion gene may act as anoncogeneto promote the development of myxoid liposarmas.[6]TheEWSR1-DDIT3fusion gene forms by a merger of theEWSR1FET gene family gene located at band 12.2 on the q arm ofchromosome 22with part of theDDIT3gene. This fusion gene is notated as t(12;22)(q13;12).[7][8][9]
Additional images
[edit]-
Micrographof myxoid liposarcoma.H&E stain -
MRIof myxoid liposarcoma of high grade, in left axillary region of 40 year old man. Horizontal section.
See also
[edit]References
[edit]- ^"Orphanet: Myxoid/round cell liposarcoma".Orpha.net.RetrievedOctober 27,2019.
- ^Sung; et al. (2000)."Myxoid Liposarcoma: Appearance at MR Imaging with Histologic Correlation".RadioGraphics.20(4): 1007–1019.doi:10.1148/radiographics.20.4.g00jl021007.PMID10903690.
- ^Flucke U, van Noesel MM, Siozopoulou V, Creytens D, Tops BB, van Gorp JM, Hiemcke-Jiwa LS (June 2021)."EWSR1-The Most Common Rearranged Gene in Soft Tissue Lesions, Which Also Occurs in Different Bone Lesions: An Updated Review".Diagnostics (Basel, Switzerland).11(6): 1093.doi:10.3390/diagnostics11061093.PMC8232650.PMID34203801.
- ^Aman P, Ron D, Mandahl N, et al. (1993). "Rearrangement of the transcription factor gene CHOP in myxoid liposarcomas with t(12;16)(q13;p11)".Genes Chromosomes Cancer.5(4): 278–85.doi:10.1002/gcc.2870050403.PMID1283316.S2CID1998665.
- ^Göransson M, Andersson MK, Forni C, et al. (January 2009). "The myxoid liposarcoma FUS-DDIT3 fusion oncoprotein deregulates NF-kappaB target genes by interaction with NFKBIZ".Oncogene.28(2): 270–8.doi:10.1038/onc.2008.378.PMID18850010.
- ^Owen I, Yee D, Wyne H, Perdikari TM, Johnson V, Smyth J, Kortum R, Fawzi NL, Shewmaker F (September 2021)."The oncogenic transcription factor FUS-CHOP can undergo nuclear liquid-liquid phase separation".Journal of Cell Science.134(17).doi:10.1242/jcs.258578.PMC8445604.PMID34357401.
- ^Rabbitts TH, Forster A, Larson R, Nathan P (1993). "Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma".Nat. Genet.4(2): 175–80.doi:10.1038/ng0693-175.PMID7503811.S2CID5964293.
- ^Crozat A, Aman P, Mandahl N, Ron D (1993). "Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma".Nature.363(6430): 640–4.Bibcode:1993Natur.363..640C.doi:10.1038/363640a0.PMID8510758.S2CID4358184.
- ^Forni C, Minuzzo M, Virdis E, et al. (February 2009)."Trabectedin (ET-743) promotes differentiation in myxoid liposarcoma tumors".Mol. Cancer Ther.8(2): 449–57.doi:10.1158/1535-7163.MCT-08-0848.PMID19190116.