Cytomorphological features of epithelioid hemangioendothelioma in ascitic fluid with radiological, clinical and histopathological correlations.

ABSTRACT

BACKGROUND: Epithelioid hemangioendothelioma (EHE) is an uncommon vascular soft-tissue tumor. Five cases of EHE in body fluids have been documented in the literature, all of them occurring in pleural effusions. This is the first description of cytomorphological features of EHE cells in ascitic fluid, accompanied by corresponding histopathological findings, clinical, and radiological data. CASE REPORT Our patient presented with several liver masses, peritoneal involvement, bilateral pleural effusions, and massive ascites. EHE was suspected on cytological examination of the ascitic fluid and was confirmed by immunohistochemical studies. Simultaneously, a liver mass was identified and diagnosed on biopsy as EHE, affording accurate histopathological correlation. Cytologically, EHE cells appear relatively bland, often obscured by reactive mesothelial cells, and dispersed singly or clustered. They often possess intracytoplasmic vacuoles, referred to as 'blister' or 'signet ring' cells. High-power examination shows slightly misshapen mildly hyperchromatic nuclei with inconspicuous nucleoli. Immunohistochemically, EHE cells express strong positivity with vascular markers (CD31, CD34 and factor VIII). They are nonreactive with mesothelial markers (calretinin and WT-1). CONCLUSION: Recognition of the possibility of EHE cells in fluid by morphology should prompt proper immunohistochemical work-up to ensure an accurate diagnosis and timely patient management.