Myxofibrosarcoma

Myxofibrosarcoma is a malignant soft tissue tumour. It is classified as a type of fibroblastic/myofibroblastic tumour and is sometimes known as a myxoid variant of malignant fibrous histiocytoma 1.

Epidemiology

Typically presents in elderly 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 extremeties.

Location

It typically occurs in the extremities. Tends to favour the dermal and subcutaneous tissues than deeper soft tissues 1,5.

Radiographic features

MRI

Myxofibrosarcoma often shows abnormal signal infiltration along the fascial plane on MRI 2.

Typical signal characteristics include:

  • T1: the myxoid component is often of low signal
  • T2/STIR: the myxoid component is of high signal
  • T1 C+ (Gd): the myxoid component tends not to enhance while the remaining soft tissue component enhances

Treatment and prognosis

Prognosis will somwaht depend on grade reference required. 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 superficial spreading configuration and can even metastasises to various distant sites 3.

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Article Information:

rID: 10772
System: Musculoskeletal
Tag: cases
Synonyms or Alternate Spellings:
  • Myxoid variant of malignant fibrous histiocytoma
  • Myxoid variant of pleomorphic undifferentiated sarcoma
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    Case 1: rare occurence in calvarium
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