Neuron-specific enolase-producing epithelioid leiomyosarcoma with gross vascular invasion and intracardiac involvement.

We present an unusual case of epithelioid leiomyosarcoma in the right shoulder. A 10-cm tumor eroding the right scapula occurred in a 59-year-old man and grew into the subclavian vein with extension to the right atrium. An elevated serum level of neuron-specific enolase was also detected. Clinical presentation suggested small cell carcinoma of the lung (Pancoast tumor) with intravenous extension. Eventually, the patient died of respiratory insufficiency. Autopsy revealed a primary tumor in the right shoulder, numerous tumor emboli in the pulmonary arteries, and associated hemorrhagic infarction. Histopathologic features revealed an epithelioid leiomyosarcoma. Furthermore, immunohistochemical and immunoblotting analyses of the tumor demonstrated positive reactions for neuron-specific enolase. We interpreted this peculiar case to be a neuron-specific enolase-producing epithelioid leiomyosarcoma of the soft tissue associated with gross vascular invasion.