Primary Pleural Epithelioid Angiosarcoma With Extensive and Rapid Metastasis to Brain and Bilateral Adrenal Glands.

Abstract

A 64-year-old male presents with shoulder pain, arm pain, and a chronic cough. CT imaging of the thorax shows a large 8.0 x 6.7 cm mass with central necrosis in the left upper lung lobe with invasion into the chest wall with partial destruction of the second and third ribs, and left axillary adenopathy. Bilateral adrenal nodules are identified via CT imaging and subsequently biopsied. Histologically, the mass reveals sheets of atypical epithelioid cells with round nuclei and abundant eosinophilic cytoplasm. Immunostaining is positive for CD31, CD34, FLI-1, AE1/AE3, and CK7, diagnostic of primary epithelioid angiosarcoma. The patient developed symptoms of confusion, dizziness, and ataxia. An MRI showed metastatic brain lesions. One month later, the patient had worsening symptoms. Repeat imaging demonstrates enlargement of the bilateral adrenal masses, a new lesion posterior to the left kidney, and doubling of the size of the brain lesions. This case illustrates the metastatic potential and pattern of the spread of an aggressive primary pleural angiosarcoma that is not described elsewhere in current literature. It also highlights the importance of timely intervention based on the rapid metastatic progression of this neoplasm.