Pleural epithelioid hemangioendothelioma.

A 51-year-old female presented to the emergency department complaining of left-sided, nonpleuritic chest pain and a 10-lb. weight loss over the previous 2 months. She was a current smoker with a 20 pack-year history. A PA/lateral chest radiograph and chest computed tomogram (CT) demonstrated a large loculated effusion with pleural thickening (Fig. 1). There were no lung nodules or pathologic mediastinal or hilar lymphadenopathy. A diagnostic thoracentesis was performed, and serosanguineous pleural fluid was obtained. Cytology was negative. For further diagnostic sampling, she underwent a thoracotomy with pleural fluid drainage and pleural biopsy, which demonstrated sheets of atypical epithelioid cells displaying prominent intra-alveolar growth (Fig. 2). The cells stained positive for CD31 and CD34. Stains for pan-cytokeratin, CK5-6, p63, calretinin, S-100, Melan-A, TTF-1, CD68, and EMA were negative. She was given a diagnosis of pleural epithelioid hemangioendothelioma. In the multidisciplinary oncology conference, it was felt she was not a candidate for surgical resection given the extensive nature of the tumor. She was offered chemotherapy with doxorubicin. Less than 4 months after her initial presentation, the patient was admitted to the hospital with a bowel obstruction. Repeat imaging demonstrated further extension of tumor in the left hemithorax with associated volume loss, an enlarging right-sided pleural effusion, evidence of liver and spleen metastasis, and new-onset ascites. Her condition continued to deteriorate with the onset of respiratory failure and she died several days later.