Skeletal Ewing sarcoma

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Skeletal Ewing sarcoma

ORPHA:319Disease

Also called Osseous Ewing sarcoma

What it is

Ewing's sarcoma is a malignant small round cell bone tumor with strong metastatic potential.

Key facts

Prevalence
1-9 / 100 000 (lifetime, Europe)
Age of onset
All ages
Inheritance
Not applicable
Classified as
Disease

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

ERGPart of a fusion gene
ETV1Part of a fusion gene
ETV4Part of a fusion gene
EWSR1Part of a fusion gene
FLI1Part of a fusion gene

ICD-10 codes

C40.0filed under a broader ICD-10 category
C40.1filed under a broader ICD-10 category
C40.2filed under a broader ICD-10 category — shared with 1 other rare disease
C40.3filed under a broader ICD-10 category
C41.2filed under a broader ICD-10 category
C41.3filed under a broader ICD-10 category
C41.4filed under a broader ICD-10 category — shared with 1 other rare disease

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

GARD 6390MEDDRA 10015560MONDO 0012817OMIM 612219UMLS C0585474

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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