Primary pleural epithelioid hemangioendothelioma with rhabdoid phenotype: report and review of the literature.
Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor described in diverse locations including lung and liver. Relative to these sites, primary EHE of the serous cavities is uncommon. EHE in the serous cavities mimics mesothelioma and adenocarcinoma clinically, radiographically, cytologically, and histologically. EHEs have plasmacytoid epithelioid cells with cytoplasmic vacuoles. In addition to these features, we noted eccentric nuclei with abundant eosinophilic cytoplasm and nuclei displaced peripherally by globular cytoplasmic inclusions imparting a "rhabdoid" phenotype. These cells were often seen surrounding a hyaline core. Rhabdoid features are not unique to a single entity, and a comprehensive immunohistochemical panel is essential. We report the occurrence of pleural EHE with rhabdoid features presenting in a pleural effusion, and review the literature of primary serosal EHEs.
Saqi
Anjali
A
Department of Pathology, New York Presbyterian Hospital, Weill Cornell Medical Center, 520 East 70th Street, New York, NY 10021, USA. aasaqi@med.cornell.edu
Nisbet
Lynn
L
Gagneja
Priti
P
Leslie
Kevin O
KO
D000230:Adenocarcinoma / Q000473:pathology; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D003922:Diabetes Mellitus, Type 1; D003937:Diagnosis, Differential; D018323:Hemangioendothelioma, Epithelioid / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008654:Mesothelioma / Q000473:pathology; D010996:Pleural Effusion / Q000166:cytology; D010997:Pleural Neoplasms / Q000378:metabolism / Q000473:pathology*
D014408:Biomarkers, Tumor