- 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.