summary Epitheliod Sarcomas are rare, slow-growing, malignant, nodular, soft tissue tumors that most commonly occur in the hand and wrist. The condition is typically seen in patients between the ages of 10 and 35 who present with a small, firm, painless, mass in the upper extremity. Diagnosis is made with a biopsy showing a characteristic nodular pattern with central necrosis within granulomatous areas. Treatment is usually wide surgical excision with radiation. Epidemiology Demographics occurs in adolescents and young adults (ages 10-35 years) 2:1 male to female ratio Anatomic location most common soft tissue sarcoma of the hand and wrist also occurs in forearm, buttock/thigh, knee, and foot mass is deep or superficial may ulcerate and mimic skin carcinoma, rheumatoid nodule, or granuloma when superficial may be attached to tendons, tendon sheaths, or fascia when deep Etiology Genetics unknown Malignancy regional lymph node metastases common systemic metastasis to lungs can occur Symptoms Symptoms small, firm, painless, slow-growing mass commonly occurring in the upper extremity Physical exam 3-6 cm firm, painless mass may have skin ulceration Imaging Radiographs calcification occurs within the lesion in 10-20% erosion of adjacent bone is sometimes found MRI indeterminate in appearance dark on T1 bright on T2 tendon sheath nodule may be visualized Histology Characteristics nodular pattern with central necrosis within granulomatous areas epithelial appearance with ovoid or polygonal cells with eosinophilic cytoplasm dense, hyalinized collagen deposits intercellularly cellular pleomorphism is rare keratin-positive staining Treatment Operative wide excision with adjuvant radiotherapy indication all operable tumors technique perform sentinel node biopsy to evaluate for regional lymph node metastasis outcomes high rate of multiple recurrences if mistaken for a benign lesion and inadequately excised re-excision of the tumor bed is recommended for inadequate initial resection amputation indications may be necessary to prevent spread of disease in cases of multiple recurrences Prognosis Extremely poor