A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology

Abstract

Malignant epithelioid hemangioendothelioma (MEHE) is a rare vascular tumor with a biological behavior that lies between those of classical epithelioid hemangioendothelioma and angiosarcoma. Furthermore, MEHE is rarely diagnosed by fine needle aspiration cytology. The authors describe the cytological features of MEHE in a 41-year-old man who presented with increasing dyspnea over a period of 1 month before admission. Computed tomography of the chest showed a 3 cm poorly defined mass in the right lower lobe. Fine needle aspiration cytology demonstrated cellular smears of loosely cohesive clusters of epithelioid cells with numerous intracytoplasmic lumens in a necrotic background. Cellular features included fine chromatin and vesicular or slightly hyperchromatic nuclei with inconspicuous nucleoli and intranuclear inclusions. Nuclear membranes were relatively irregular with indentation. Mean N/C ratio was not increased, presumably due to a moderate amount of cytoplasm. The histologic examination displayed epithelioid and spindle cell proliferation with necrosis accompanying a classical epithelioid hemangioendotheliomatous area. The immunohistochemical evaluation was confirmatory and showed immunoreactivity for vascular markers. The authors also reviewed FNAB findings of epithelioid angiosarcoma, primary adenocarcinoma, and bronchioloalveolar carcinoma of the lung to identify cytomorphologic differences by literature bases. MEHE of the lung is difficult to diagnose cytologically because of its rarity and its cytomorphologic similarities with other malignant epithelial and mesenchymal tumors. However, it may be possible to distinguish it from other entities when the possibility of this unusual vascular neoplasm is suspected and ancillary studies are supportive.

Epithelioid hemangioendothelioma in pleural effusion

“…5,13,14 The fine-needle aspiration biopsy of malignant mesothelioma may contain epithelioid cell clusters with little atypia and papillary formation like EHE. Adenocarcinoma is positive for CEA, Leu M1, and HMFG-2.…”

Cytopathologic Characteristics of Epithelioid Vascular Malignancies

“…Most FNA descriptions of EAS and EHE in the literature are case reports or small case series [15,16,17,18,19,20,21,22,23,24,25], with the exception of one larger case series on EHE [26]. A cytomorphological study conducted by Liu and Layfield [27] described common features found in 11 AS, but this study did not exclusively examine EAS.…”

Cytomorphological Features of Epithelioid Hemangioendothelioma in Ascitic Fluid with Radiological, Clinical and Histopathological Correlations

“…Epithelioid hemangioendothelioma (EHE) was first characterized by Weiss and Enzinger [1 ]in 1982 as a low-grade angiosarcoma. Due to its epithelioid morphology and ‘signet ring' cell configuration, EHE may mimic various pathological entities such as mesothelioma, adenocarcinoma, melanoma, and fibrous histiocytoma [2,3,4]. The cytomorphology of EHE specimens from fine-needle aspirates of solid tumors has been well described [4,5,6,7,8,9], but the cytological features of these tumors in effusions are limited to descriptions of only 5 cases, all of which are pleural fluids.…”

“…Due to its epithelioid morphology and ‘signet ring' cell configuration, EHE may mimic various pathological entities such as mesothelioma, adenocarcinoma, melanoma, and fibrous histiocytoma [2,3,4]. The cytomorphology of EHE specimens from fine-needle aspirates of solid tumors has been well described [4,5,6,7,8,9], but the cytological features of these tumors in effusions are limited to descriptions of only 5 cases, all of which are pleural fluids. Clinically aggressive types of EHE tend to involve the pleura and peritoneum [10], and the exfoliated tumor cells account for those found in effusions.…”