Dedifferentiated liposarcoma

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Dedifferentiated liposarcoma

ORPHA:99970Histopathological subtype

Also called DDLS

What it is

Dedifferentiated liposarcoma (DDLS) is a high-grade subtype of liposarcoma (LS) that progresses from well-differentiated liposarcoma (WDLS), and most often occurs in the retroperitoneum. It is defined as a region of nonlipogenic sarcoma associated with WDLS. .

Key facts

Prevalence
1-9 / 1 000 000 (annual incidence, Europe)
Age of onset
Adult
Inheritance
Not applicable
Classified as
Histopathological subtype

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes

CDK4Role in the phenotype of
HMGA2Role in the phenotype of
MDM2Role in the phenotype of

ICD-10 codes

C49.9filed under a broader ICD-10 category — shared with 24 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MEDDRA 10073135MONDO 0020563UMLS C0205824

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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