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Myofibroblastic malignancies.

Cyril Fisher1

  • 1Department of Histopathology, Royal Marsden Hospital, London, UK. cyril.fisher@rmh.nthames.nhs.uk

Advances in Anatomic Pathology
|June 29, 2004
PubMed
Summary
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Myofibrosarcomas are malignant tumors of myofibroblasts, with low-grade variants resembling fibrosarcoma and high-grade types linked to malignant fibrous histiocytoma. Their classification and distinct genetic features are reviewed.

Area of Science:

  • Oncology
  • Pathology
  • Soft Tissue Neoplasms

Background:

  • Malignant tumors of myofibroblasts (myofibrosarcomas) are increasingly recognized but remain controversial.
  • Existing examples include spindle cell or pleomorphic soft tissue neoplasms with varying histological grades.

Purpose of the Study:

  • To review the concept of myofibrosarcoma.
  • To describe the different variants of myofibrosarcoma.

Main Methods:

  • Histological grading of myofibrosarcomas.
  • Immunohistochemical marker expression analysis.
  • Differential diagnosis consideration with benign proliferations and other sarcomas.

Main Results:

  • Low- and intermediate-grade myofibrosarcomas are fascicular, deep soft tissue neoplasms (often head/neck) that can recur locally but rarely metastasize.

Related Experiment Videos

  • High-grade (pleomorphic) myofibrosarcomas are a subset of malignant fibrous histiocytoma.
  • Distinct genetic findings differentiate inflammatory myofibroblastic tumor and infantile fibrosarcoma.

    • Conclusions:

    • Myofibrosarcoma classification encompasses a spectrum of histological grades and behaviors.
    • Accurate diagnosis requires distinguishing these malignant tumors from benign myofibroblastic proliferations and other sarcomas.