Sclerosing Epithelioid Fibrosarcoma



Sclerosing Epithelioid Fibrosarcoma


Cyril Fisher, MD, DSc, FRCPath









Hematoxylin & eosin shows nests of cells with clear cytoplasm and ovoid, occasionally folded nuclei in a fibrous stroma. Ultrastructurally, these cells show features of fibroblasts.






Hematoxylin & eosin shows cords or files of polygonal cells, some with eosinophilic cytoplasm, infiltrating dense fibrous stroma. This can mimic carcinoma, notably lobular carcinoma of the breast.


TERMINOLOGY


Definitions



  • Fibrosarcoma variant characterized by at least focal epithelioid cytomorphology and areas of dense fibrosis


ETIOLOGY/PATHOGENESIS


Environmental Exposure



  • Occasional example attributed to therapeutic irradiation


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare: ˜ 60 examples reported


  • Age



    • Adults, peak in 5th decade


  • Gender



    • Slightly more common in females


Site



  • Limbs and limb girdles, trunk, shoulder, neck



    • Rarely in visceral sites, e.g., large intestine, ovary, base of penis, pituitary


  • Deep muscle



    • Around periosteum or fascia


  • Can involve or arise in bone including skull, rib, sacrum


Presentation



  • Deep mass


  • Painful or painless mass


Treatment



  • Surgical approaches



    • Adequate local excision



      • Can require amputation especially when bone involved


  • Adjuvant therapy



    • Chemotherapy or radiation therapy can be tried to control recurrence and metastasis


Prognosis



  • Histologically of variable grade but clinically aggressive, especially in long term



    • Local recurrence in > 50%


    • Metastasis in 43-86%


    • 5-year survival is 43-75%


MACROSCOPIC FEATURES


General Features



  • In deep soft tissue


  • Multinodular


  • Circumscribed or infiltrative


  • Very hard, can show calcification or ossification


  • Occasionally cystic


Sections to Be Submitted



  • Lesion should be extensively sampled to detect cellular areas


Size



  • Variable



    • 2 to > 20 cm in maximum dimension


MICROSCOPIC PATHOLOGY


Histologic Features

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Sclerosing Epithelioid Fibrosarcoma
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