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Overall, the 10-year survival rate of MLS individuals has been 77%, a survival rate appreciably longer than other liposarcoma forms. Compared to low-risk MLS, high-risk MLS (risk defined by tumor round cell content and/or other unfavorable prognostic indicators) is associated with increased rates of metastasis and therefore a shorter survival time.
A myxoid liposarcoma is a malignant adipose tissue neoplasm [1] of myxoid appearance histologically.. Myxoid liposarcomas are the second-most common type of liposarcoma, representing 30–40% of all liposarcomas in the limbs, occurring most commonly in the legs, particularly the thigh, followed by the buttocks, retroperitoneum, trunk, ankle, proximal limb girdle, head and neck, and wrist.
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.
EMC was firstly described in 1953 by Stout et al. when they discussed the different species of extraskeletal chondrosarcoma, [2] but EMC concept was firstly proposed in 1972 by Enzinger et al. [3] Brody thought that this was a unique low-grade malignancy with a low growth rate and both clinically and histopathologically distinct anamnesis beside the typical chondrosarcomas. [4]
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 ]
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Post-treatment local recurrences and metastases were observed in 15% and 38% of cases; 5- and 10-year overall survival rates were 60% and 48%, respectively; Overall median survival time were 10.1 years; and patients with tumors ≥10 cm in longest diameter had an almost 6-fold higher rate of developing metastases than patients with tumors 4 cm ...
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