epithelioid sarcoma
epithelioid sarcoma
epithelioid sarcoma
A rare, low-grade sarcoma, which primarily affects the distal extremities, especially the hand and fingers. Male:female ratio, 2:1, affecting patients age 10–35 if distal and older if proximal.DiffDx-benign
Benign fibrous histiocytoma, fibromatosis, granuloma annulare, infectious granuloma, myositis ossificans, necrobiosis lipoidica, necrobiotic granuloma, nodular fasciitis, rhabdoid tumour, rheumatoid nodule.
DiffDx-malignant
Adnexal carcinoma, clear-cell carcinoma, epithelioid vascular neoplasm, fibrosarcoma, melanoma, metastatic carcinoma, squamous cell carcinoma, synovial sarcoma.
Management
Wide local excision or amputation.
Prognosis
Recurrence in 35–75%; 40–45% metastasise—e.g., to lung, regional lymph nodes, scalp; 5-year survival in 80%; 10-year survival in 50%.
Adverse prognosticators
Deep, > 5 cm, high mitotic rate, vessel and nerve invasion, recurrence, nodal metastasis.
Epithelioid sarcoma types
Conventional (diffuse) form
Affects distal extremities, especially hand and fingers; male:female ratio, 2:1; affects patients age 10–35 if distal and older if proximal.
Proximal type
Older patients; more aggressive than conventional (distal) type; located deep in the pelvis, perineum, pubis, vulva and penis.
Micro
Large epithelioid cells with marked atypia and prominent nucleoli, varying amounts of rhabdoid features.