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
  • UMLS:C0023269 (E (Exact mapping: the two concepts are equivalent))
  • 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).)
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: