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Dedifferentiated liposarcoma (DDLS) is a high-grade subtype of liposarcoma (LS; see this term) that progresses from well-differentiated liposarcoma (WDLS; see this term), and most often occurs in the retroperitoneum. It is defined as a region of nonlipogenic sarcoma associated with WDLS. .
ORPHA:99970Classification level: Subtype of disorder
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Adult
- ICD-10: C49.9
- OMIM: -
- UMLS: C0205824
- MeSH: -
- GARD: -
- MedDRA: -
The incidence is approximately 1/ 330, 000 persons/year.
DDLS is a fast growing tumor with potential for distant metastasis but this occurs in less than 15% of patients. Like WDLS, it is usually a painless mass, though it can cause symptoms of obstruction, decrease in appetite and abdominal distention.
The etiology is unknown. Like WDLS, DDLS usually has amplification of the chromosome region 12q and associated amplification and overexpression of the MDM2, HMGA2 and CDK4 genes.
The 5-year disease-specific survival rate for DDLS overall is 44% but this is location dependent with extremity lesions having a 5-year disease-specific survival rate of greater than 90%.
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