A rare soft tissue sarcoma characterized by a malignant, fibroblastic lesion with variably myxoid stroma, pleomorphism, and a distinctively curvilinear vascular pattern. The majority of tumors arise in the limbs including the limb girdles, more often in dermal/subcutaneous tissues than in the underlying fascia and skeletal muscle, and usually present as a slowly growing, painless mass. Depth of the lesion and tumor grade do not influence the high rate of local recurrence, while the percentage of metastasis and tumor-associated mortality are much higher in deep-seated and high-grade neoplasms.

Synonyms: Myxoid malignant fibrous histiocytoma Fibromyxosarcoma

This is just here as a test because I lose it

Term information

database cross reference
  • MedDRA:10066948 (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:C3714524 (E (Exact mapping: the two concepts are equivalent))
expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=79105

hasDbXref

ICD-10:C49.9

MedDRA:10066948

UMLS:C3714524

has age of onset

http://www.orpha.net/ORDO/Orphanet_409948

has inheritance

http://www.orpha.net/ORDO/Orphanet_409941

has point prevalence range

http://www.orpha.net/ORDO/Orphanet_409981

notation

ORPHA:79105

part of

http://www.orpha.net/ORDO/Orphanet_206982

http://www.orpha.net/ORDO/Orphanet_3394

present in

http://www.orpha.net/ORDO/Orphanet_409991