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Alveolar soft tissue sarcoma

Disease definition

A rare soft tissue sarcoma characterized by a slowly growing, painless space-occupying lesion, composed of large, uniform, epithelioid cells arranged in solid nests and/or alveolar structures, separated by thin, sinusoidal vessels. The tumor mostly affects adolescents and young adults. Early metastasis, most commonly to the lung, bones, and brain, is a characteristic feature and relevant prognostic factor, together with age at presentation and tumor size, while histological features have no prognostic significance.


Classification level: Disorder
  • Synonym(s):
    • ASPS
    • Alveolar soft part sarcoma
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: C49.9
  • ICD-11: XH8V95
  • OMIM: 606243
  • UMLS: C0206657
  • MeSH: D018234
  • GARD: 5654
  • MedDRA: 10001882

Detailed information


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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