myxoid liposarcoma

PO

Definition: Myxoid liposarcoma displays variably aggressive behavior and responds poorly to available systemic therapies.

Images

 myxoid liposarcoma

• https://twitter.com/histiocytosisX/status/748157284644196352
• https://twitter.com/a_sleeping_dog/status/773402045911007232
• https://twitter.com/JMGardnerMD/status/737075990086590466
• https://twitter.com/DanglisFotiosMD/status/734976870110900225
• https://twitter.com/solcosao/status/706608037868404736

  FISH FUS rearrangement

• https://twitter.com/histiocytosisX/status/748157284644196352

 myxoid liposarcoma with cystic spaces

• https://twitter.com/chioseasi/status/697807147695009793

Immunochemistry

 detection of TLS-EWSR1 or TLS-DDIT3 ( FUS-CHOP ) chimeric oncoprotein by monoclonal antibody(16538055)

Cytogenetics

 t(12;16)(q13;p11) creating a TLS-DDIT3 fusion gene ( FUS-CHOP fusion gene)

Molecular biology

 TLS-DDIT3 fusion gene (FUS-CHOP fusion gene) by t(12;16)(q13;p11)
 EWSR1-CHOP fusion gene (12169678, 16884691)

 detection of TLS-EWSR1 or TLS-DDIT3 ( FUS-CHOP ) chimeric oncoprotein by monoclonal antibody (16538055)

Expression studies

 Expression levels of the ret proto-oncogene (RET ), insulin-like growth factor 1 receptor (IGFR1 ), and insulin-like growth factor 2 (IFG2 ) correlate with poor metastasis-free survival, supporting a role for ERK/MAPK and PI3K/AKT pathways in clinically aggressive myxoid liposarcomas. (19368956)

See also

 adipocytic tumors

• liposarcomas
  • myxoid/round cell liposarcoma (MRC-LPS) : FUS rearranged
  • WD/DD liposarcoma (WDDD-LPS) : CDK4/MDM2 amplified

Open access references

 Detection of myxoid liposarcoma-associated FUS-DDIT3 rearrangement variants including a newly identified breakpoint using an optimized RT-PCR assay. Powers MP, Wang WL, Hernandez VS, Patel KS, Lev DC, Lazar AJ, López-Terrada DH. Mod Pathol. 2010 Oct;23(10):1307-15. PMID: 20581806 [Free]

References

 Validation of immature adipogenic status and identification of prognostic biomarkers in myxoid liposarcoma using tissue microarrays. Cheng H, Dodge J, Mehl E, Liu S, Poulin N, van de Rijn M, Nielsen TO. Hum Pathol. 2009 Apr 13. PMID: 19368956

 Matsui Y, Ueda T, Kubo T, Hasegawa T, Tomita Y, Okamoto M, Myoui A, Kakunaga S, Yasui N, Yoshikawa H. A novel type of EWS-CHOP fusion gene in myxoid liposarcoma. Biochem Biophys Res Commun. 2006 Sep 22;348(2):437-40. PMID: 16884691

 Oikawa K, Ishida T, Imamura T, Yoshida K, Takanashi M, Hattori H, Ishikawa A, Fujita K, Yamamoto K, Matsubayashi J, Kuroda M, Mukai K. Generation of the novel monoclonal antibody against TLS/EWS-CHOP chimeric oncoproteins that is applicable to one of the most sensitive assays for myxoid and round cell liposarcomas. Am J Surg Pathol. 2006 Mar;30(3):351-6. PMID: 16538055

 Crozat A, Aman P, Mandahl N, Ron D. Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature. 1993 Jun 17;363(6430):640-4. PMID: 8510758

 Mrozek, K.; Karakousis, C. P.; Bloomfield, C. D. : Chromosome 12 breakpoints are cytogenetically different in benign and malignant lipogenic tumors: localization of breakpoints in lipoma to 12q15 and in myxoid liposarcoma to 12q13.3. Cancer Res. 53: 1670-1675, 1993. PubMed ID : 8453640

 Rabbitts, T. H.; Forster, A.; Larson, R.; Nathan, P. : Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma. Nature Genet. 4: 175-180, 1993. PubMed ID : 7503811

 Aman, P.; Ron, D.; Mandahl, N.; Fioretos, T.; Heim, S.; Arheden, K.; Willen, H.; Rydholm, A.; Mitelman, F. : Rearrangement of the transcription factor gene CHOP in myxoid liposarcomas with t(12;16)(q13;p11). Genes Chromosomes Cancer 5: 278-285, 1992. PubMed ID : 1283316