Lung
Mesenchymal tumors
Epithelioid hemangioendothelioma
Author: Deepali Jain, M.D.
Last author update: 1 September 2012
Last staff update: 1 October 2020
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: pulmonary epithelioid hemangioendothelioma
Table of Contents
Definition / general | Case reports | Gross description | Microscopic (histologic) description | Positive stains | Electron microscopy description | Differential diagnosisCite this page: Jain D. Epithelioid hemangioendothelioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungtumorEHE.html. Accessed December 25th, 2024.
Definition / general
- Formerly called intravascular bronchioloalveolar tumor (see also Soft tissue topic)
- 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
Microscopic (histologic) 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
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
