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.

Scanning magnification 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.



  • Epithelioid sarcoma (ES)


  • Epithelioid cell sarcoma (no longer recommended)


  • 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


Genetic Factors

  • Some cases have abnormalities of chromosome 22q

  • Rare association with neurofibromatosis type 2



  • 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

    • Usually young, but may occur at any age

    • Classic ES

      • Mostly 2nd to 4th decades

    • Proximal ES

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

  • Gender

    • More frequent in males


  • 1 or more slow-growing lesions

  • Subcutaneous mass

    • Classic ES

      • Dermal or subcutaneous nodule

    • Proximal ES

      • Subcutaneous or deeper mass

      • Can appear more rapidly

  • Ulcer

    • Nonhealing ulcer with raised margin

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 tumor with high mortality


  • Surgical approaches

    • Adequate local excision

      • Amputation for intractable recurrences

  • Adjuvant therapy

    • No specific effective therapy


  • 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

    • Greater degree of necrosis

    • Vascular invasion

    • Inadequate local excision


General Features

  • Classic ES

    • Ulcerated skin nodule

    • Raised “sealing-wax” margins

  • Proximal ES

    • Multinodular mass

    • Hemorrhage and necrosis


  • Classic ES: 0.2 cm to > 5 cm diameter

  • Proximal ES: Up to 20 cm diameter


Histologic Features

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