Epithelioid Hemangioendothelioma in Pleural Effusion Maryam Sayah, MD, 1 Christopher VandenBussche, MD, PhD, 2 and Zahra Maleki, MD 2 * Epithelioid hemangioendothelioma (EHE) is a rare mesenchy- mal tumor of vascular origin with variable morphological fea- tures and unpredictable biological behavior. EHE occasionally involves the pleural fluid. However, the cytomorphology of EHE found in pleural fluid has not been well characterized in the lit- erature. Herein we describe a case of EHE, initially presenting as multiple liver lesions plus several small nodular densities at the bases of the lungs followed by bilateral pleural effusions. Diagn. Cytopathol. 2015;43:751–755. V C 2015 Wiley Periodicals, Inc. Key Words: pleural effusion; cytology; epithelioid heman- gioendothelioma; vascular neoplasm Epithelioid hemangioendothelioma (EHE) is a rare mes- enchymal tumor of vascular origin with variable morpho- logical features and unpredictable biological behavior. In 1982, EHE was first described by Weiss and Enzinger as a low-grade angiosarcoma. 1 While these lesions are some- times diagnosed on the fine-needle aspiration of a solid lesion, EHE occasionally involves the pleural fluid. How- ever, the cytomorphology of EHE found in pleural fluid has not been well characterized in literature. Here we describe a case of EHE, initially presenting as multiple liver lesions plus several small nodular densities at the bases of lungs, followed by bilateral pleural effusions. Case Report A 20-year-old female presented with severe right upper quadrant pain, nausea, and vomiting. Physical examina- tion and laboratory findings were otherwise unremarkable. An MRI of the abdomen showed four lesions in the right lobe of the liver ranging from 1.2 to 3 cm in size. A sub- sequent percutaneous liver biopsy revealed EHE. Her work up also included CT of thorax, which revealed small nodular densities approximately 8 mm in diameter at the bases of lungs, most consistent with metastatic dis- ease. She underwent chemoembolization; however, further subsequent imaging studies showed that her lesions were unchanged in size. One year later, the patient’s MRI revealed an increase in the size of the lung lesions with several new satellite lesions. She underwent additional chemoembolization and her disease remained stable for 9 years. After this period of time, she developed a large left pleural effusion with near-total consolidation of the lung plus multiple expan- sile metastases involving the eighth rib. Three months later, she was admitted for worsening dyspnea at both rest and with minimal exertion. A chest CT showed a left hydropneumothorax and small right pleural effusion. A left-sided pleural tube was placed which initially drained 200 cc of fluid. This fluid was sent to the cytopathology laboratory for analysis. Three slides were prepared including two cytospins and one cell block which subsequently stained with Papanico- laou stain and H&E, respectively. The Pap stain slides showed numerous neoplastic cells in clusters or individu- ally in a background of lymphocytes and mesothelial cells. The neoplastic cells were intermediate to large as compared to mesothelial cells. The cells had high nuclear-to-cytoplasmic ratio. The nuclei were centrally located and they exhibited coarse granular chromatin, one or two nucleoli, and irregular nuclear membrane (Table I). Binucleation, multinucleation, and mitotic figures were common findings. Occasional nuclear grooves were noted while no cytoplasmic intranuclear pseudoinclusions were seen. Scattered plasmacytoid cells with eccentric nuclei 1 University of Texas Southwestern Medical Center, Dallas, Texas 2 The Johns Hopkins Hospital, Baltimore, Maryland Dr Maryam Sayah is an adjunct instructor. Dr Christopher VandenBussche is an assistant professor. Dr Zahra Maleki is an assistant professor. The authors have no financial interest or conflicts of interest to disclose. *Correspondence to: Z. Maleki, MD, Assistant Professor of Pathology, The Johns Hopkins Hospital, Department of Pathology/Division of Cytology, 600 N. Wolfe Street/Carnegie 469C, Baltimore, MD 21287, USA. E-mail: zmaleki1@jhmi.edu Received 4 March 2015; Revised 29 April 2015; Accepted 22 June 2015 DOI: 10.1002/dc.23315 Published online 17 July 2015 in Wiley Online Library (wileyonlinelibrary.com). V C 2015 WILEY PERIODICALS, INC. Diagnostic Cytopathology, Vol. 43, No 9 751