Myxofibrosarcoma is a malignant soft tissue tumour. It is classified as a type of a fibroblastic/myofibroblastic tumour.
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Terminology
Previously known as a myxoid variant of malignant fibrous histiocytoma 7.
Epidemiology
Typically presents in older patients (6th decade) 3. There may be male predilection. Although rare in general, it is considered the most common soft tissue tumour to present in late adult life.
Clinical presentation
Patients often present with a painless enlarging soft tissue mass in the extremities.
Pathology
Location
Tends to favour the dermal and subcutaneous tissues than deeper soft tissues 1,5,7:
- lower limbs (~77%)
- trunk (~12%)
- retroperitoneum/mediastinum (~8%)
- head and neck (3-10%)
Macroscopic appearance
In contrast to most other histologic types of low-grade soft-tissue sarcoma, recurrent low-grade myxofibrosarcomas are often infiltrative, shows tapering, have tail-like margins with a superficial spreading configuration, and can even metastasise to various distant sites 3.
Radiographic features
MRI
Myxofibrosarcoma often shows abnormal signal infiltration along the fascial plane on MRI 2. There may be a fascial tail sign.
- T1: the myxoid component is often of low signal
- T2/STIR: the myxoid component is high signal
- T1 C+ (Gd): the myxoid component tends not to enhance while the remaining soft tissue component enhances
Treatment and prognosis
Prognosis will somewhat depend on grade 7.
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