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Disease definition

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.


Classification level: Disorder
  • Synonym(s):
    • Fibromyxosarcoma
    • Myxoid malignant fibrous histiocytoma
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: C49.9
  • ICD-11: 2B53.0
  • OMIM: -
  • UMLS: C3714524
  • MeSH: -
  • GARD: -
  • MedDRA: 10066948

Detailed information

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