Epithelioid Sarcoma



Epithelioid Sarcoma


Cyril Fisher, MD, DSc, FRCPath









Clinical photograph shows several rounded lesions with raised red margins and central “targetoid” ulceration on the forearm of a 23-year-old man. Nonhealing ulcers are typical of epithelioid sarcoma.






Hematoxylin & eosin shows skin with an irregularly shaped dermal lesion. Note the cellular rim image and the necrobiotic central zone. The epidermis shows thinning and ulceration image over the lesion.


TERMINOLOGY


Abbreviations



  • Epithelioid sarcoma (ES)


Synonyms



  • Epithelioid cell sarcoma (no longer recommended)


Definitions



  • Malignant mesenchymal tumor resembling carcinoma or granuloma, which shows predominantly epithelial but also mesenchymal differentiation


  • ES occurs in classical and proximal (aggressive, large cell, or rhabdoid) forms


ETIOLOGY/PATHOGENESIS


Genetic Factors



  • Some cases have abnormalities of chromosome 22q


  • Rare association with neurofibromatosis type 2


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare



      • Accounts for about 1% of all soft tissue sarcomas


    • Classic ES



      • Most common in distal extremities, especially hand and forearm


      • Head and neck


      • Penis, vulva


    • Proximal ES



      • Proximal limb girdle


      • Axial locations: Perineum, pelvis, mediastinum


      • Trunk: Chest wall


  • Age



    • Any age


    • Classic ES



      • Mostly 2nd-4th decades


    • Proximal ES



      • Median age 40 years (range 13-80 years)


  • Gender



    • More frequent in males


Presentation



  • Slow growing


  • Subcutaneous mass


  • Ulcer



    • Classic ES



      • Dermal or subcutaneous nodule


      • Nonhealing ulcer


    • Proximal ES



      • Subcutaneous or deep mass


      • Can appear more rapidly


Natural History



  • Classic ES



    • Persistent and multiple recurrences



      • Successive lesions often recur and extend more proximally in limb


    • Eventual metastasis



      • To regional lymph nodes


      • Via blood to lungs, bone, brain, and soft tissue, notably scalp


  • Proximal ES



    • Rapidly growing, locally aggressive mass with high mortality


Treatment



  • Options, risks, complications



    • Mainly surgical


  • Surgical approaches



    • Adequate local excision



      • Amputation for intractable recurrences


  • Adjuvant therapy



    • No specific effective therapy


Prognosis



  • Classic ES




    • > 70% recur


    • 30-50% metastasize


    • 5-year survival (70%), 10-year survival (40%)


  • Proximal ES



    • 65% local recurrence


    • 45-75% metastasize


    • 5-year survival (35-65%)


  • Favorable prognostic factors



    • Young age at 1st diagnosis


    • Female sex


    • Primary tumor < 2 cm diameter


  • Adverse prognostic factors



    • Proximal location


    • Amount of necrosis


    • Vascular invasion


    • Inadequate local excision


MACROSCOPIC FEATURES


General Features



  • Classic ES



    • Ulcerated skin nodule


    • Raised “sealing-wax” margins


  • Proximal ES



    • Multinodular mass


    • Hemorrhage and necrosis


Size



  • Classic ES: 0.2 cm to > 5 cm diameter


  • Proximal ES: Up to 20 cm diameter


MICROSCOPIC PATHOLOGY


Histologic Features

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