Myxofibrosarcoma

Orpha code: 79105OMIM code:

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.

Disease data
Classification

Disease

Synonyms
Fibromyxosarcoma
Śluzakowata złośliwa włóknista histiocytoma
Włókniakośluzakomięsak
Myxoid malignant fibrous histiocytoma
ORPHA code
79105
OMIM code
-
ICD10 code
C49.9
ICD11 code
2B53.0

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl