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.


Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, June 20, 2012 3:05 pm