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Myofibroblastic Sarcoma

DEFINITION AND PATHOGENESIS

IMPORTANCE

  •  Propensity for local recurrence and associated with a low risk of metastatic spread

CLINICAL FEATURES

RADIOLOGIC FEATURES

  • Radiographs may reveal osseous erosion
  • MRI
    • Hypointense on T1
    • Hyperintense on T2
  • FDG-PET
    • SUV reported 9.8
      • Found incidentally using FDG-PET as a cancer screening tool

GROSS PATHOLOGY

  •  Firm pale white mass with ill-defined margins

HISTOLOGIC/MOLECULAR FEATURES

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Nodular fasciitis
  • Fibrosarcoma
  • Aggressive fibromatosis
  • Calcifying fibrous pseudotumor
  • Inflammatory myofibroblastic tumor
  • Epithelioid sarcoma
  • Myofibroblastic differentiation of other sarcomas

DISEASE COURSE AND TREATMENT

SPECIAL CONSIDERATION

  • Fibroblastic and Myofibroblastic Sarcoma

    • Spindle-cell sarcoma showing fibroblastic-myofibroblastic differentiation with a predominance of fibroblasts has been reported in the skin
  • High-grade Myofibroblastic Sarcoma

    • Has been reported arising from the pericardium
      • LR treated with chemotherapy (doxorubicin/ifosfamide) and XRT with 6 month disease free survival reported
    • Has been reported associated with XRT (postradiation high-grade myofibroblastic sarcoma)
  • Epithelioid Inflammatory Myofibroblastic Sarcoma

    • See Inflammatory Myofibroblastic Tumor (IMT)
  • Vaccination Associated Myofibroblastic Sarcoma

    • Reported 4 months after Rabies vaccination with multiple LRs
  • Animal Model

    • Has been reported in the maxillary region in a dog
    • Has been reported in the orbit of a cat
    • Has been reported at the second site of an equine influenza vaccination in a quarterhorse shortly after the vaccination
      • Associated with multifocal osseous metaplasia
    • Has been reported in the pleura of a pig

REFERENCES

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