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Lung tumor

Other malignancies

Epithelioid hemangioendothelioma

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 22 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Formerly called intravascular bronchioloalveolar tumor
● 80% women, usually young adults
● Neoplastic, but usually not metastatic
● Progressive growth, usually remains within thoracic cavity, may cause death from respiratory insufficiency
● Other sites: liver, bone
● 10% have peripheral eosinophilia
● Poor prognosis if vascular spread, pleural involvement, severe symptoms

Case reports

● 54 year old man with multiple pulmonary nodules (Arch Pathol Lab Med 2003;127:e319)

Gross description

● Multiple round, well demarcated nodules < 2 cm, often in lower lung, with a gray-white peripheral rim and chondroid appearance
● Resembles metastasis, granulomatous disease or interstitial lung disease
● May spread along pleura or pericardium and resemble mesothelioma

Micro description

● Central hyalinized stroma, eosinophilic amorphous material or coagulative necrosis with variable calcification surrounded by thin rim of plump eosinophilic endothelial cells
● Clusters fill alveoli, apparently through pores of Kohn, and occasionally bronchioles, arteries, veins
● Nuclei are bland, round/oval, may have cytoplasmic vacuoles
● No/minimal mitoses
● Lung architecture preserved

Micro images

H&E, Factor VIII (figure D)

Positive stains

● CD10 (Arch Pathol Lab Med 2009;133:1965); Factor VIII, CD31, CD34
● Variable ER and PR

Electron microscopy description

● Endothelial features: well developed basal lamina, pinocytotic vesicles, occasional Weibel-Palade bodies

Differential diagnosis

● Metastatic tumor from liver or other sites: destroy lung architecture, high mitotic rate
Sclerosing hemangioma: destroys lung architecture, negative for vascular markers

End of Lung tumor > Other malignancies > Epithelioid hemangioendothelioma

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