Myxofibrosarcoma

Myxofibrosarcoma is a malignant soft tissue tumour. It is classified as a type of fibroblastic/myofibroblastic tumour.

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%)
Histology

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 metastasize to various distant sites 3.

Radiographic features

MRI

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

Signal characteristics
  • 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 somewhat depend on grade 7

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Article Information

rID: 10772
System: Musculoskeletal
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Myxoid variant of malignant fibrous histiocytoma
  • Myxoid variant of pleomorphic undifferentiated sarcoma

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