Epithelioid hemangioendothelioma is a low-grade malignant vascular
tumour that was previously considered an intermediate vascular
tumour (between hemangioma and conventional angiosarcoma), but was
reclassified as a low-grade angiosarcoma in the 2002 WHO
Pulmonary epithelioid hemangioendothelioma (PEH) is the current
term for a rare neoplasm originally described by Dail and Liebow
in 1975 as intravascular sclerosing bronchioalveolar tumour of the
Development of immunohistochemical techniques confirmed its
endothelial lineage, and Wiess et
al. subsequently suggested the current name, "epithelioid
epithelioid hemangioendothelioma, typically manifests as multiple
bilateral lung nodules that are usually discovered incidentally in
young or middle-aged women, although cases in children and the
elderly have also been reported.
Symptomatic patients , presence of cough, hemoptysis, chest pain,
multiple unilateral nodules, pleural effusion, metastases to more
than one site and lymph node metastases are the factors associated
with a poor prognosis.
(6) Most commonly, PEH
presents as bilateral or unilateral multiple pulmonary nodules with
clear boundaries, diameters of 0.3–2 cm, pale gray or brown
coloration and these can invade the pleura and cause pleural
effusion or nodular thickening.
features are epithelioid tumour cells with
nests, cords and strands embedded in a sclerosing, hyaline matrix with
alveolar, bronchiolar and vascular extension.
Areas of necrosis and ossification
may be present and the mitotic index is usually below
one mitosis in 10 HPF.
examination of the tumour shows
positivity for CD31, CD34 and most of the tumours are positive for factor VIII,
and negative for pancytokeratin and calretinin.
(10) Due to the fact
that PEH is rare, clinically, it is easily confused with a variety of
benign and malignant lung diseases.
diagnosis of PEH includes chronic granulomatous disease, amyloid
nodules, hamartoma, primary or metastatic lung cancer, malignant
mesothelioma and angiosarcoma.