loading...| Preferred Name |
Alveolar soft tissue sarcoma |
|
| Synonyms |
Alveolar soft part sarcoma |
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| Definitions |
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. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_163699 |
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| alternative_term |
Alveolar soft part sarcoma ASPS |
|
| 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. |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=163699 |
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| hasDbXref |
OMIM:606243 ICD-10:C49.9 MedDRA:10001882 UMLS:C0206657 MeSH:D018234 |
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| label |
Alveolar soft tissue sarcoma |
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| notation |
ORPHA:163699 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_163699 |
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| prefLabel |
Alveolar soft tissue sarcoma |
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| treeView | ||
| subClassOf |
| Delete | Mapping To | Ontology | Source |
|---|---|---|---|
| http://purl.bioontology.org/ontology/RCD/Xa99f | RCD | LOOM | |
| http://www.gamuts.net/entity#alveolar_soft_part_sarcoma | GAMUTS | REST |