Disease relevance of Liposarcoma, Myxoid

• Two distinct FUS breakpoint clusters in myxoid liposarcoma and acute myeloid leukemia with the translocations t(12;16) and t(16;21) [1].
• Of particular interest is the frequent involvement of the 12q13-14 segment in clonal abnormalities in lipomas and myxoid liposarcomas, and it is possible that LRP may play a role in the pathogenesis of such tumors [2].
• This tumor should be differentiated from myxoid chondrosarcoma, myxoid liposarcoma, chondromyxoid fibroma, and metastatic chordoma [3].
• The staining reaction of intramuscular myxoma and myxoid liposarcoma corresponded to that of synovial fluid and cock's comb (containing hyaluronic acid) [4].
• C/EBPzeta, also known as GADD153, CHOP10, and DDIT3 has been found associated with the development of myxoid liposarcoma and the progression of melanoma [5].

High impact information on Liposarcoma, Myxoid

• In human myxoid liposarcoma, a chromosomal rearrangement leads to fusion of the growth-arresting and DNA-damage-inducible transcription factor CHOP (GADD153) to a peptide fragment encoded by the TLS gene [6].
• The TLS-CHOP oncoprotein, found in the majority of human myxoid liposarcomas, consists of a fusion between the transcription factor CHOP/GADD153 and the N terminus of an RNA-binding protein TLS/FUS [7].
• However, myxoid liposarcoma DNA samples contained altered restriction fragments detectable with GLI probes that were highly specific and reproducible from case to case [8].
• Fusion of the EWS and CHOP genes in myxoid liposarcoma [9].
• Prognostic impact of P53 status, TLS-CHOP fusion transcript structure, and histological grade in myxoid liposarcoma: a molecular and clinicopathologic study of 82 cases [10].

Chemical compound and disease context of Liposarcoma, Myxoid

• As a tumor progression model, we investigated 26 myxoid liposarcomas (11 pure myxoid grade I, 15 myxoid/round cell grade II/III) for the hTERT expression level and compared the results of the new method with former measurements performed in silver-stained polyacrylamide gels [11].
• Although a tentative diagnosis of poorly differentiated myxoid liposarcoma was made, ultrastructural examination and Oil Red O fat stain failed to demonstrate the evidence of lipoblastic differentiation, except that occasional cells possessed a small number of fine fat droplets [12].
• Gadolinium-enhanced imaging is important in differentiating myxoid liposarcoma from benign cystic tumors [13].
• Myxoid liposarcomas had a high water content and contained considerable amounts of free cholesterol and phospholipids [14].
• The responsiveness to trabectedin in type II myxoid liposarcoma xenografts was as high as in patients [15].

Biological context of Liposarcoma, Myxoid

• The locus GLI, which encodes a zinc-finger protein, has been mapped to the same region as the myxoid liposarcoma breakpoint [8].
• TLS was originally identified as part of a fusion protein with CHOP arising from chromosomal translocation in human myxoid liposarcomas [16].
• Null mdm2 immunophenotype, coupled with evidence of a specific chromosome translocation t(12;16), was constantly observed in both the usual and the cellular subtypes of myxoid liposarcoma, three cases of which also showed TP53 alterations at the genetic or protein level [17].
• Myxoid liposarcoma with adipocytic maturation: detection of TLS/CHOP fusion gene transcript [18].
• RNA splicing mediated by YB-1 is inhibited by TLS/CHOP in human myxoid liposarcoma cells [19].

Anatomical context of Liposarcoma, Myxoid

• An abnormal extra nuclear localization of PML bodies was also found in TLS-CHOP carrying cell lines established from myxoid liposarcomas [20].
• Elevated hTERT expression (cut-off ratio x 100 at 1.3) was an indicator of round cell components and hence for tumor progression in myxoid liposarcoma [11].

Gene context of Liposarcoma, Myxoid

• To examine whether telomerase activity, hTRT and hTR mRNA expression were associated with tumor progression in myxoid liposarcoma, we investigated a total of 28 myxoid liposarcomas (13 pure myxoid tumors, 14 mixed-type tumors, and 1 pure round-cell variant) from 19 patients [21].
• Expression of DOL54 is not restricted to myxoid liposarcomas with the FUS-DDIT3 chimera but is found in various sarcomas [22].
• The 16p11 breakpoint in myxoid liposarcomas might affect the expression of the LRP gene on 16p11.2 encoding the multidrug resistance associated major vault protein [23].
• Cases of purely low-grade myxoid liposarcoma (16 cases) were characterised by low-cellularity, mucin pooling and plexiform vasculature [24].
• The myxoid liposarcoma specific TLS-CHOP fusion protein localizes to nuclear structures distinct from PML nuclear bodies [20].

References