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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.

ORPHA:163699

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

  A summary on this disease is available in Español (2020) Français (2020) Nederlands (2020)

Detailed information

Guidelines

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

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.