Sarcoma low-malignant fibromyxoides C49.9

Evans 1993

Low malignant, fibroblastically differentiated new formation, which can occur as a blurred, nodular 2.0-26 cm large tumor mass in the deep soft tissue, but also dermal-subcutaneously. Postoperatively, the tumor tends to recur, which can occur even after years of latency.

The incidence was 0.18/1 million people according to a Danish study. m:f=4:6

Children and more often adolescents and younger adults.

Subfascial on extremities/trunk (90%), rarely head and neck. Dermal-subcutaneous regions are rarely included.

Tumor nodes consisting of EMA-, MUC4-, and also alpha-smooth muscle actin positive spindle tumor cells are characterized by alternating arc-shaped fascicles with myxoid sections. Tumour cells with cytological atypia, also epithelioid aspects and mitoses. A traslocation t(7;16)(q33;p11) leading to a fusion gene (FUS-CREB3L2) can be detected.

Excision with sufficient safety distance.

Metastasis risk is about 10%.

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