A rare soft tissue sarcoma characterized by a malignant space-occupying lesion most commonly located in the retroperitoneum or the inferior vena cava, but also other soft tissues, and composed of cells showing distinct features of smooth muscle cells. The tumor presents with mass effect depending on the location. It is capable of both local recurrence and distant metastasis, while lymph node metastasis is rare. Prognosis largely depends on tumor location and size.

This is just here as a test because I lose it

Term information

database cross reference
  • MedDRA:10024189 (E (Exact mapping: the two concepts are equivalent))
  • MeSH:D007890 (E (Exact mapping: the two concepts are equivalent))
  • ICD-10:C49.9 (- NTBT (ORPHA code's Narrower Term maps to a Broader Term). - Attributed (The ICD10 code is attributed by Orphanet).)
  • UMLS:C0023269 (E (Exact mapping: the two concepts are equivalent))
expertlink
  • https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=64720
hasDbXref
  • MeSH:D007890
  • ICD-10:C49.9
  • UMLS:C0023269
  • MedDRA:10024189
has age of onset
  • http://www.orpha.net/ORDO/Orphanet_409949
  • http://www.orpha.net/ORDO/Orphanet_409948
  • http://www.orpha.net/ORDO/Orphanet_409947
  • http://www.orpha.net/ORDO/Orphanet_409946
has inheritance
  • http://www.orpha.net/ORDO/Orphanet_409941
notation
  • ORPHA:64720
part of
  • http://www.orpha.net/ORDO/Orphanet_3394
  • http://www.orpha.net/ORDO/Orphanet_289656

Term relations

Subclass of: