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Myxofibrosarcoma
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.
ORPHA:79105
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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- Clinical practice guidelines
- English (2018)
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