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Epithelioid Sarcoma

- Discussion:
    - epithelioid sarcoma is the most common soft-tissue sarcoma in the hand and most commonly occurs in young adults;
    - slow growing malignant soft tissue tumor, usually occuring in adults;
    - these are usually minimally symptomatic.
    - "pseudocapsule" or poorly circumscribed mass often attached to tendons, tendon sheath, or joint capsule;
    - tumors can be superficial and may become ulcerated;
    - deeper lesions are often attached to tendons, tendon sheaths, or fascial structures

- Sites of Involvement:
    - predisposition for fingers, hands, and forearm;
    - most common soft tissue sarcoma of hand
    - may present as a small ulcerating nodule on the extensor surface of hand;

- Histology:
    - may mimic a necrotizing granulomatous process.
    - usually there are chronic inflammatory cells along the margin of the tumor nodules (nodular pattern with central necrosis)
    - microscopic exam reveals different types of mono or biphasic cells
    - may be a mix of spindle and epithelial cells;

- Prognosis and Treatment:
    - despite its slow growth, the aggressive nature of tumor is evidence that 85% recurrence after local excision and 30% have metastasized;
    - note that epithelioid sarcoma can spread via lymph nodes, which impacts on prognosis;
           - other sarcomas which spread via lymph nodes include: clear cell sarcoma, synovial sarcoma,  rhabdomyosarcoma;
    - recommended treatment is radical resection or amputation;
    - this is an aggressive tumor which may be found to metastasize even after years have passed

Epithelioid sarcoma in the upper extremity.

Epithelioid sarcoma. An analysis of fifty-one cases.

Primary epitheliod sarcoma of the hand and forearm: a review of 13 cases

Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma.

The Doctor's Doctor. Epitheloid Sarcoma.