Alveolar soft tissue sarcomaGo to external page http://www.orpha.net/ORDO/Orphanet_163699
A rare soft tissue sarcoma characterized by a slowly growing, painless space-occupying lesion, composed of large, uniform, epitheloid 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.
Synonyms: ASPS Alveolar soft part sarcoma
- UMLS:C0206657 (E (Exact mapping: the two concepts are equivalent))
- MedDRA:10001882 (E (Exact mapping: the two concepts are equivalent))
- MeSH:D018234 (E (Exact mapping: the two concepts are equivalent))
- ICD-10:C49.9 (- NTBT (ORPHA code's Narrower Term maps to a Broader Term). - Index term (The ORPHA code is listed in the ICD10 Index).)
- OMIM:606243 (E (Exact mapping: the two concepts are equivalent))