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Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of low-grade sarcoma first described by H. L. Evans in 1987. [1] LGFMS are soft tissue tumors of the mesenchyme-derived connective tissues; on microscopic examination, they are found to be composed of spindle-shaped cells that resemble fibroblasts. [2]
Of particular importance, the presence of pseudo-lipoblasts in a myxoid sarcoma-like background is an extremely strong indicator that the tumor is a MFS. [ 6 ] and tumors with a myxofibrosarcoma-like histopathology that initiate in the retroperitoneum, abdominal cavity, or pelvis are nearly always dedifferentiated liposarcomas .
[9] 3) express a FUS-CREB3L2 fusion gene is more likely to be a low-grade fibromyxoid sarcoma than a SEF (i.e. the FUS-CREB3L2 fusion gene is present in >90% of low-grade fibromyxoid sarcomas but only ~20% of SEF tumor cells [17]); or 4) express an EWSR1-CREB3L1 or EWSR1-CREB3L2 fusion gene is likely to be a SEF tumor. [14]
Low-grade myofibroblastic sarcoma [32] Superficial CD34-positive fibroblastic tumour [33] Myxoinflammatory fibroblastic sarcoma, [34] also termed acral myxoinflammatory fibroblastic sarcoma because it was initially thought to be limited to acral (i.e. leg and arm) areas. [35]
Differential diagnosis includes periungual and subungual fibroma, acquired digital fibrokeratoma, superficial angiomyxoma, myxoid neurofibroma, dermal mucinosis, sclerosing perineuroma, low grade fibromyxoid sarcoma, myxoid dermatofibrosarcoma protuberans, fibroma of tendon sheath, giant cell tumour, glomus tumour, fibrous histiocytoma ...
Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. Fibrosarcomas mainly arise in people between the ages of 25 and 79. [1]
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