- 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.
- 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;
-
- 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. RS Bryan, EH Soule, and JH Dobyns. JBJS Vol 56-A. p 458-465. 1974.
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