Details of Disease

General Information of Disease (ID: DISXU8CC)

Disease Name	Pleomorphic sarcoma
Synonyms	PLLS; pleomorphic liposarcoma (morphologic abnormality); PLS; pleomorphic liposarcoma
Disease Class	2B54: Pleomorphic sarcoma
Definition	Pleomorphic liposarcoma (PLS), the rarest subtype of liposarcoma (LS), is an aggressive, fast growing tumor located usually in the deep soft tissues of the lower and upper extremities. It is characterized by a variable number of pleomorphic lipoblasts and, in contrast to dedifferentiated liposarcoma, it lacks any association with well-differentiated liposarcoma.
Disease Hierarchy	DIS8IZVM: Liposarcoma DISXU8CC: Pleomorphic sarcoma
ICD Code	ICD-11 ICD-11: 2B54 ICD-9 ICD-9: 171 Expand ICD-11 'XH0947 Expand ICD-9 171
Disease Identifiers	MONDO ID MONDO_0020562 MESH ID D008080 UMLS CUI C0205825 MedGen ID 61457 Orphanet ID 99969 SNOMED CT ID 112683004

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
KN035	DMKLMG6	Phase 2	Antibody	[1]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 4 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
PLIN1	TTIV27N	moderate	Biomarker	[2]
CDK4	TT0PG8F	Strong	Biomarker	[3]
CPM	TTVOH2M	Strong	Biomarker	[4]
FASN	TT7AOUD	Strong	Therapeutic	[5]
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This Disease Is Related to 4 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CBX3	OTOP9RLD	Strong	Altered Expression	[6]
NF1	OTC29NHH	Strong	Biomarker	[7]
NNAT	OTNRLO7G	Strong	Altered Expression	[8]
DDIT3	OTI8YKKE	Definitive	Genetic Variation	[9]
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References

1	ClinicalTrials.gov (NCT04480502) ENVASARC: Envafolimab And Envafolimab With Ipilimumab In Patients With Undifferentiated Pleomorphic Sarcoma Or Myxofibrosarcoma (ENVASARC). U.S. National Institutes of Health.
2	Perilipin 1 Expression Differentiates Liposarcoma from Other Types of Soft Tissue Sarcoma.Am J Pathol. 2019 Aug;189(8):1547-1558. doi: 10.1016/j.ajpath.2019.04.017. Epub 2019 May 22.
3	Primary dermal pleomorphic liposarcoma: utility of adipophilin and MDM2/CDK4 immunostainings.J Cutan Pathol. 2017 Mar;44(3):283-288. doi: 10.1111/cup.12850. Epub 2016 Nov 16.
4	Liposarcomas with mixed well-differentiated and pleomorphic features: a clinicopathologic study of 12 cases.Am J Surg Pathol. 2010 Jun;34(6):837-43. doi: 10.1097/PAS.0b013e3181dbf2f7.
5	Fatty acid synthesis is a therapeutic target in human liposarcoma. Int J Oncol. 2010 May;36(5):1309-14. doi: 10.3892/ijo_00000616.
6	CBX3 predicts an unfavorable prognosis and promotes tumorigenesis in osteosarcoma.Mol Med Rep. 2019 May;19(5):4205-4212. doi: 10.3892/mmr.2019.10104. Epub 2019 Mar 28.
7	Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms.Acta Neuropathol. 2014 Apr;127(4):565-72. doi: 10.1007/s00401-014-1246-6. Epub 2014 Jan 25.
8	Integrative DNA methylation and gene expression analysis in high-grade soft tissue sarcomas.Genome Biol. 2013 Dec 17;14(12):r137. doi: 10.1186/gb-2013-14-12-r137.
9	Analysis of CHOP rearrangement in pleomorphic liposarcomas using fluorescence in situ hybridization.Cancer Sci. 2009 Jan;100(1):82-7. doi: 10.1111/j.1349-7006.2008.01008.x. Epub 2008 Nov 24.