[7] Rarely, these tumors have presented in the breast, heart, paratesticular region (i.e. area inside the scrotum including the epididymis the spermatic cord along with its coverings),[8] eye,[9] bone, liver, or multiple sites concurrently.
[12] MFS tumors often infiltrate along vascular and fascial planes, are incompletely removed at surgery, and consequently recur at the surgical site.
[13] Recurrences of the MFS at the site of surgery have developed in 16% to 57% of patients with a significant proportion (25%–52%) recurring multiple times.
Lower-grade MFS tissues consist of scattered large, variability-sized and spindle-shaped-to-variably-shaped tumor cells with darkly stained nuclei.
[4] Higher grade FBS tumors consist of relatively large sheets of these spindle-shaped/vatiably-shaped cells in a similar myxoid background containing thin-walled curvilinear blood vessels.
[7] 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.
On T2-weighted MRI, 81% of MFS tumors give a tail sign, i.e. a multidirectional signal spreading away from the main mass along a facial plane (i.e. a line or band of connective tissue).
This MRI finding is also extremely valuable for gauging the extent and depth of surgery needed to completely remove MBS tumors.
This is done to ensure that all tumor tissue is removed in order to avoid the high risks of local recurrences and worsening prognoses.
[7] Current drug therapies that are or may soon be tried in treating MFS include angiogenesis inhibitors and immunotherapeutics such as Bevacizumab and Nivolumab.