Myxofibrosarcoma



Myxofibrosarcoma


David Cassarino, MD, PhD










Grossly, myxofibrosarcoma often shows a multinodular growth pattern with gelatinous-appearing cut surfaces. (Courtesy T. Mentzel, MD.)






Scanning magnification view shows a superficial low-grade myxofibrosarcoma involving the subcutis and deep dermis. Numerous elongated, thin vessels image are easily identified in a prominent myxoid stroma.


TERMINOLOGY


Abbreviations



  • Myxofibrosarcoma (MFS)


Synonyms



  • Myxoid malignant fibrous histiocytoma (MFH)


Definitions



  • MFS represents a spectrum of malignant fibroblastic neoplasms with variably myxoid stroma and characteristic elongated curvilinear vessels


CLINICAL ISSUES


Epidemiology



  • Incidence



    • One of most common sarcomas in elderly patients


  • Age



    • Affects mainly patients in 6th to 8th decade


    • Exceptionally rare in patients < 20 years old


  • Gender



    • Slight male predominance


Site



  • Majority arise in limbs and limb girdles



    • Lower > upper extremities


  • Rarely on trunk, head and neck region


  • Very rarely on hands and feet


  • 2/3 of cases arise in dermal/subcutaneous tissues


Presentation



  • Painless mass


  • Slow growing


Treatment



  • Surgical approaches



    • Complete and wide excision


Prognosis



  • Local, often repeated, recurrences in up to 50-60% of cases (unrelated to histologic grade)


  • Low-grade MFS usually does not metastasize


  • Low-grade MFS may show tumor progression in subsequent recurrences


  • Intermediate- and high-grade MFS may develop metastases in 30-35% of cases


  • Overall 5-year survival is 60-70%


MACROSCOPIC FEATURES


General Features



  • Superficially located neoplasms consist of multiple variably gelatinous or firmer nodules


  • Deep-seated neoplasms often present as single mass with myxoid cut surfaces


  • Areas of tumor necrosis may be seen in high-grade neoplasms

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Myxofibrosarcoma
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