Pulmonary epithelioid hemangioendothelioma

Last revised by Yuranga Weerakkody on 26 Jul 2021

Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of the lung and/or pleura with low malignant potential.

It is a rare tumor, with ~50 cases reported. Patient age at presentation ranges from 25-54 years old, with a female predilection.

Pulmonary epithelioid hemangioendothelioma is often asymptomatic and discovered incidentally. It has been associated with chest tightness and shortness of breath.

The exact etiology of pulmonary epithelioid hemangioendothelioma is unclear. It may occur in male patients, which confuses the usual explanation of a role of estrogen in tumor formation.

Histology shows epithelioid or spindle-shaped cells with abundant eosinophilic cytoplasm. Lumina or vacuoles containing erythrocytes are present. Cells are arranged in nests and cords with degenerated interstitial mucoid. 

Usually discovered incidentally.

  • multiple nodules in the lungs or pleura
    • bilateral, random distribution
    • nodules usually 0.3-2.0 cm (although up to 5 cm has been reported)
  • calcifications are occasionally seen within the nodules
  • if pleural invasion, then pleural thickening or effusion may occur

The prognosis of pulmonary epithelioid hemangioendothelioma is unpredictable due to variable estrogen effects on the tumor. Life expectancy has ranged from 1-25 years, depending on presentation and possible resectability of the lesions. If the lesions are unresectable, then chemotherapy is usually used. 

  • male gender
  • hemoptysis
  • asymmetric distribution
  • pleural effusions

Originally described in 1975 as an "intravascular sclerosing bronchioloalveolar tumor (IVBAT) of the lung".

The imaging differential is wide, including:

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Cases and figures

  • Case 1: pulmonary epithelioid hemangioendothelioma
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  • Case 2:hepatic and pulmonary epithelioid hemangioendothelioma
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