Epithelioid sarcomas are the most common soft tissue sarcoma of the hand. These lesions are rare, malignant, slow-growing soft-tissue tumors. They are often misdiagnosed as benign cutaneous or subcutaneous lesions or ulceration that can masquerade as an infectious process. They are commonly located in the upper extremities of young adults and have a tendency for local recurrence. Epithelioid tumors frequently metastasize to the lungs and lymph nodes.1-6
Pathophysiology
- Tumor of lineage and uncertain differentiation
- Displays carcinoma-like epithelial differentiation
- Predominantly solid, multinodular mass with multiple areas of hemorrhage and necrosis
- Nodules in deep soft tissue, less frequently in skin or subcutis
Related Anatomy
- Predominantly on forearm, dorsum of the hand, palm or finger. 6
Incidence and Related Conditions
- More common in men than in women (2:1)
- Estimated annual incidence of upper extremity epithelioid sarcoma was 0.4 cases per 1 million individuals in 2005
- Metastasizes in about 40% of patients to the lungs (predominantly), lymph nodes, bone and brain
Differential Diagnosis 1-6
- Chronic granulomatous inflammation
- Fibrous histocytoma
- Nodular fasciitis
- Fibromatosis
- Giant cell tumor of tendon sheath
- Melanoma
- Clear-cell sarcoma of the tendon and aponeurosis
- Schwannoma
- Metastatic squamous cell carcinoma
- Metastatic adenocarcinoma
- Synovial sarcoma
- Epithelioid hemagnioendothelioma
- Epithelioid leiomyosarcoma
- Malignant extrarenal rhabdoid tumors
- Epithelioid vascular neoplasm
- Amelanotic melanoma
- Angiosarcoma
- Fibrosarcoma
- Malignant fibrous histiosarcoma
- Low grade chronic infection
- Verruca vulgaris (wart)
- Unhealed wound